3D printed temporal bone as a tool for otologic surgery simulation.

PURPOSE: In this face validity study, we discuss the fabrication and utility of an affordable, computed tomography (CT)-based, anatomy-accurate, 3-dimensional (3D) printed temporal bone models for junior otolaryngology resident training. MATERIALS AND METHODS: After IRB exemption, patient CT scans were anonymized and downloaded as Digital Imaging and Communications in Medicine (DICOM) files to prepare for conversion. These files were converted to stereolithography format for 3D printing. Important soft tissue structures were identified and labeled to be printed in a separate color than bone. Models were printed using a desktop 3D printer (Ultimaker 3 Extended, Ultimaker BV, Netherlands) and polylactic acid (PLA) filament. 10 junior residents with no previous drilling experience participated in the study. Each resident was asked to drill a simple mastoidectomy on both a cadaveric and 3D printed temporal bone. Following their experience, they were asked to complete a Likert questionnaire. RESULTS: The final result was an anatomically accurate (XYZ accuracy = 12.5, 12.5, 5 µm) 3D model of a temporal bone that was deemed to be appropriate in tactile feedback using the surgical drill. The total cost of the material required to fabricate the model was approximately $1.50. Participants found the 3D models overall to be similar to cadaveric temporal bones, particularly in overall value and safety. CONCLUSIONS: 3D printed temporal bone models can be used as an affordable and inexhaustible alternative, or supplement, to traditional cadaveric surgical simulation.

Rate of tympanic membrane perforation after intratympanic steroid injection.

PURPOSE: To determine the rate of persistent tympanic membrane perforation after intratympanic steroid injection. To determine which comorbid conditions and risk factors are associated with prolonged time to perforation closure following intratympanic steroid injection. MATERIALS AND METHODS: Clinical data were gathered for patients who had undergone intratympanic steroid injection to treat sudden sensorineural hearing loss or Ménière's disease. Primary outcomes analysis included rate of persistent tympanic membrane perforation, defined as perforation at least 90days following last injection, and time to perforation healing. Age, sex, number of injections, smoking status, diabetes mellitus, previous head and neck irradiation, and concurrent oral steroids, were analyzed as potential predictors of persistent perforation. RESULTS: One hundred ninety two patients were included in this study. Three patients (1.6%) had persistent tympanic membrane perforations. All three patients received multiple injections. One patient underwent tympanoplasty for repair of persistent perforation. The median time to perforation healing was 18days. There was no statistically significant variable associated with time to perforation healing. However, patients with prior history of head and neck radiation averaged 36.5days for perforation healing compared to 17.5days with no prior history of radiation and this approached statistical significance (p=0.078). CONCLUSIONS: The rate of persistent tympanic membrane perforation following intratympanic steroid injection is low. Patients with a history of radiation to the head and neck may be at increased risk for prolonged time for closure of perforation.

Factors associated with recurrence of squamous cell carcinoma involving the temporal bone.

OBJECTIVE: This study aimed to better identify factors associated with recurrence of squamous cell carcinoma (SCC) involving the temporal bone. METHODS: A retrospective study was conducted at a tertiary hospital. Sixty patients who were diagnosed over a 10-year period with SCC involving the temporal bone and underwent surgical resection were analyzed. All patients were staged based on the University of Pittsburgh staging system. Demographic, intraoperative, and pathologic data were analyzed with respect to recurrence. RESULTS: Thirteen (21.7%) patients were T1, 8 (13.3%) T2, 7 (11.7%) T3, and 32 (53.3%) T4. Eighteen patients (30.0%) recurred in the study period. The mean time to recurrence was 5.8 months. Tumors originating in the skin overlying the parotid gland and the external auditory canal had higher recurrence rates than those from the auricle/postauricular skin and temporal bone (P = .05). Direct parotid and perineural spread accounted for 15.0% of all routes of temporal invasion but resulted in 22.2% of all recurrences (P = .04). Increased N stage was statistically associated with increased risk of recurrence (P = .01). Cervical, as compared to perifacial and parotid, lymph node involvement was associated with increased risk of recurrence (odds ratio = 6.91; 95% confidence interval, 1.11-42.87). CONCLUSION: We have identified multiple factors that are associated with increased recurrence of SCC involving the temporal bone.

Middle ear meningiomas: a case series reviewing the clinical presentation, radiologic features, and contemporary management of a rare temporal bone pathology.

Meningiomas are the most common extra-axial intracranial neoplasm and frequently develop in the parasagittal region. Rarely, meningiomas may involve the middle ear and mastoid, resulting from contiguous spread of adjacent intracranial tumor, or less commonly as an isolated primary tumor of the middle ear. Patients with primary middle ear meningiomas (MEMs) often present with non-specific otologic complaints including hearing loss, otorrhea and otalgia thereby mimicking common chronic otitis media, while secondary lesions more frequently manifest sensorineural hearing loss, cranial neuropathy and other neurologic symptoms from the associated intracranial component. The radiological appearance of MEMs often overlaps with other tumors of the temporal bone. Therefore, a correct diagnosis cannot always be made prior to surgical biopsy. While gross total resection with preservation of existing neurological function is possible with smaller lesions, complete tumor removal may be extremely morbid with more extensive or adherent MEMs. In such cases, aggressive subtotal resection with close radiologic follow-up should be considered. Given the rarity of the studied condition, the literature addressing MEMs is sparse. The current study reviews ten additional cases of MEMs, highlighting the clinicopathologic and radiological features that distinguish meningiomas from other middle ear and mastoid pathology.

Clinical characteristics associated with isolated unilateral utricular dysfunction.

PURPOSE: The ocular vestibular evoked myogenic potential (oVEMP) represents the sound-induced activation of extraocular muscles and is believed to originate from the utricle and superior vestibular nerve. Isolated unilateral oVEMP abnormalities with otherwise normal balance function test (BFT) results have not yet been characterized in a large patient series, and their clinical significance remains unclear. MATERIALS AND METHODS: Retrospective review of adult patients with vestibular complaints at a tertiary academic neurotologic referral center was performed. Patients with isolated unilateral oVEMP abnormalities were identified. The prevalence of vestibular symptoms and results of the Dizziness Handicap Inventory (DHI) and Hospital Anxiety and Depression Scale (HADS) were compared between these patients and those with normal BFT results. RESULTS: Thirty-one adult patients with isolated unilateral oVEMP abnormalities were identified (71% female, mean age 48 ± 14 years). Presenting complaints included vertigo in 53%, non-vertiginous dizziness in 68%, postural instability in 52%, and swaying/rocking sensation in 13%. Significant differences were observed in the percentage of patients with postural instability (p=0.046) and swaying/rocking sensation (p=0.04) when comparing the abnormal oVEMP group to patients with a normal BFT battery. No differences were observed when comparing other symptoms, age, gender, diagnoses, and DHI/HADS scores between groups. CONCLUSION: This is the largest series to date reporting on patients with isolated unilateral oVEMP abnormalities. Our results suggest this population may demonstrate an increased prevalence of postural instability and swaying/rocking sensation. Other measures of postural stability may further characterize the vestibular impairments associated with isolated unilateral utricular dysfunction.

Does Compliance or Cost Regulate Effectiveness of Notched Sound Therapy Phone Applications for Tinnitus Relief? Preliminary Data of a Prospective Randomized Control Trial.

OBJECTIVE: The objective of this study is to evaluate compliance with and effectiveness of notched sound therapy (NST) administered through a mobile application in improving symptoms of patients experiencing chronic tinnitus. STUDY DESIGN: A prospective randomized control trial was done. SETTING: The study was done at a tertiary referral center. PATIENTS: Adult patients with tinnitus were monitored. INTERVENTIONS: Patients were randomized at enrollment to either the NST or a standard of care (SOC) group. NST arm included, in addition to SOC, a free subscription to NST mobile application, whereas the SOC arm included NST at subscription cost. MAIN OUTCOME MEASURES: The main outcome measures were continued enrollment in study, compliance with the therapy, frequency and duration of therapy use, and change from the baseline in the Tinnitus Handicap Inventory (THI). RESULTS: Patients in the NST group were 2.25 times more likely to use the NST application for 3 months postenrollment. Only 33% of users in the NST group listened for the prescribed 2 h/d compared with 0% of users in the SOC group. There was a clinically relevant mean decrease in THI from a baseline of 13.5 in the NST group (p = 0.09) and of 14.8 in the SOC group (p = 0.02). There was a positive correlation between initial THI and decrease in THI after 3 months (p = 0.001). CONCLUSIONS: Monitoring tinnitus for 3 months leads to a decrease in subjective symptoms regardless of NST use. Patients who received a free subscription to the application were more likely to continue with therapy, but very few patients were able to comply with 2 hours of listening time per day.

Pintail Comb Injury as a Rare Cause of Penetrating Temporal Bone Trauma With Facial Paralysis.

We present a case of novel penetrating temporal bone trauma with a pintail comb causing facial paralysis. We describe a 42-year-old woman with acute facial paralysis, hearing loss, and dizziness following accidental tympanic membrane puncture. The patient underwent middle ear exploration with tympanoplasty and was found to have an intact but severely edematous facial nerve. The patient demonstrated less than 90% degeneration under electroneuronography and was treated medically without decompression, and by 6 months had exhibited complete resolution of facial nerve dysfunction with normal hearing. This case report highlights a unique cause of penetrating temporal bone trauma and supports the utility of electroneuronography in predicting the likelihood of recovery and need for decompression in patients where the facial nerve has obvious signs of trauma but remains grossly intact.

Adenomatous Neuroendocrine Tumors of the Middle Ear: A Multi-institutional Investigation of 32 Cases and Development of a Staging System.

OBJECTIVE: To date, less than 150 cases of middle ear adenomatous neuroendocrine tumors (MEANTs) have been reported in the English literature. The objective of this study was to provide a contemporary analysis of these rare lesions and develop a consensus-driven staging system. STUDY DESIGN: Multi-institutional retrospective histopathologic, radiologic, and clinical review. SETTING: Six tertiary referral centers. PATIENTS: Thirty-two patients with pathologically confirmed MEANT. INTERVENTION: Surgical resection, adjuvant therapy. MAIN OUTCOME MEASURES: Clinical manifestations, outcomes, staging system. RESULTS: Patients commonly presented with progressive conductive or mixed hearing loss (90%), aural fullness (50%), and tonal tinnitus (46%). Pulsatile tinnitus (16%), carcinoid syndrome (4%), and facial nerve paresis (4%) were less commonly observed. MEANTs frequently mimicked temporal bone paraganglioma (31%) and cholesteatoma (15%) at presentation. According to a novel T/N/M/S staging system (S = secretory tumor) proposed herein, there were 6 (19%) T1, 19 (59%) T2, and 7 (22%) T3 MEANTs. T3 tumors were significantly more likely to undergo subtotal or near total resection compared with lower staged tumors secondary to adherence to critical neurovascular structures such as the petrous internal carotid artery and facial nerve (p = 0.027). Patients with T3 MEANTs were more likely to experience multiple recurrences, require adjuvant therapy with a somatostatin analogue, or develop permanent facial nerve paresis compared with lower staged tumors. At last follow up, no patients with T1 MEANTs had developed recurrence, whereas 37% (7/19) of patients with T2 MEANT and 57% (4/7) of patients with T3 MEANT experienced either disease recurrence after gross total resection (GTR) or regrowth of known residual tumor requiring additional surgery at a median duration of 72 months (95% CI, 24-84). CONCLUSIONS: Patients with MEANTs can present with nonspecific symptomatology that overlaps with more commonly encountered middle ear lesions. MEANT exhibits a proclivity for recurrence according to T-stage and long-term clinical follow up is necessary, particularly for advanced stage tumors.

Adenomatous tumors of the middle ear.

Adenomatous tumors are an uncommon cause of a middle ear mass. Clinical findings may be nonspecific, leading to difficulties in differentiation from other middle ear tumors. Controversy also exists whether to classify middle ear adenoma and carcinoid as separate neoplasms, or alternatively within a spectrum of the same pathologic entity. Most adenomatous middle ear tumors are indolent in behavior, with a benign histologic appearance and slowly progressive growth. The mainstay of treatment is complete surgical resection, which affords the greatest likelihood of cure.

Glomus tympanicum: a review of 115 cases over 4 decades.

OBJECTIVE: To characterize the clinical presentation, surgical management, and outcomes of a large consecutive cohort of patients with glomus tympanicum (GT) tumors managed at a single tertiary referral group over 4 decades. STUDY DESIGN: Retrospective review. SETTING: Tertiary neurotological referral center. SUBJECTS AND METHODS: All patients underwent surgical treatment of histopathologically confirmed GT between January 1973 and March 2014. Audiometric outcomes were reported according to AAO-HNS guidelines, and tumor stage was described using the Glasscock-Jackson classification system. RESULTS: There were 115 patients (90.4% women; mean age, 55.2 years) who met the inclusion criteria; 38 (33.0%) cases of GT were stage I, 51 (44.3%) stage II, 10 (8.7%) stage III, and 16 (13.9%) stage IV. There were 108 (93.9%) patients who underwent gross total removal, while 7 (6.1%) received less than complete resection for advanced disease that was adherent to the petrous carotid artery, facial nerve, stapes footplate, or round window. Two patients who underwent gross total resection experienced transient facial paresis, and 1 had internal carotid injury with stroke. No patients had been diagnosed with recurrent disease at a mean follow-up of 30.4 months. CONCLUSION: Surgery remains the treatment of choice for GT, providing a high rate of tumor control and resolution of aural symptoms with a low risk of complications. The surgical approach and extent of resection should be tailored to the patient. Gross total resection can be performed in over 90% of patients; however, leaving a limited adherent tumor remnant on the facial nerve or carotid artery should be considered with advanced infiltrative disease to prevent unnecessary morbidity.

Fibrous dysplasia of the temporal bone: a review of 66 cases.

OBJECTIVES/HYPOTHESIS: Fibrous dysplasia is a condition of nonmalignant osseous change and may occur in a monostotic or polyostotic pattern, the latter potentially being associated with McCune-Albright syndrome. Symptoms are highly variable and dependent upon lesion location and size. STUDY DESIGN: Retrospective review. METHODS: Consecutive subjects with fibrous dysplasia of the temporal bone were evaluated between 2000 and 2013 at two tertiary academic referral centers. Main outcome measures included disease presentation, diagnostic evaluation, management strategy, and outcome. RESULTS: Sixty-six patients with fibrous dysplasia of the skull were found to have involvement of the temporal bone. The mean age at diagnosis was 25 years, 39 (59%) were female, and the mean duration of follow-up was 48 months. Six (11%) patients had monostotic disease, with the remaining 60 (89%) patients having the polyostotic form; 16 (24%) patients had McCune-Albright syndrome. The most common presenting complaint was headache (59%), followed by hearing loss (29%). The most common exam finding was cosmetic deformity (50%). Cholesteatoma (3%) and spontaneous cerebrospinal fluid fistula (1.5%) were found in a small percentage. No patients had evidence of motor cranial neuropathy by history or physical examination. CONCLUSIONS: The clinical presentation of fibrous dysplasia involving the temporal bone is variable. A growing number of patients are diagnosed incidentally through imaging, and since most patients experience a benign course, the majority can be followed clinically without need for intervention. LEVEL OF EVIDENCE: 4.

Implantable hearing devices: the Ototronix MAXUM system.

For many hearing-impaired individuals, the benefits of conventional amplification may be limited by acoustic feedback, occlusion effect, and/or ear discomfort. The MAXUM system and other implantable hearing devices have been developed as an option for patients who derive inadequate assistance from traditional HAs, but who are not yet candidates for cochlear implants. The MAXUM system is based on the SOUNDTEC Direct System technology, which has been shown to provide improved functional gain as well as reduced feedback and occlusion effect compared to hearing aids. This and other implantable hearing devices may have increasing importance as future aural rehabilitation options.

Historical development of active middle ear implants.

Active middle ear implants (AMEIs) are sophisticated technologies designed to overcome many of the shortcomings of conventional hearing aids, including feedback, distortion, and occlusion effect. Three AMEIs are currently approved by the US Food and Drug Administration for implantation in patients with sensorineural hearing loss. In this article, the history of AMEI technologies is reviewed, individual component development is outlined, past and current implant systems are described, and design and implementation successes and dead ends are highlighted. Past and ongoing challenges facing AMEI development are reviewed.

Evolving considerations in the surgical management of cholesteatoma in the only hearing ear.

OBJECTIVE: To describe a contemporary, pragmatic approach to managing cholesteatoma in the only hearing ear. STUDY DESIGN: Retrospective case series. SETTING: Single tertiary referral center. PATIENTS: All patients that underwent cholesteatoma surgery, having profound hearing loss in the contralateral ear. INTERVENTION(S): Cholesteatoma surgery. MAIN OUTCOME MEASURE(S): Surgical strategy, preoperative and postoperative audiometric outcomes, short- and long-term complications, recidivism. RESULTS: Twenty-eight patients met criteria, representing 0.25% of all chronic ear surgeries performed between 1970 and 2012. Patients undergoing surgery in the latter half of the study underwent intact canal wall procedures and ossicular chain reconstruction more frequently despite having similar severities of disease. All patients with inner ear fistula underwent an open-cavity operation. In the early postoperative period, 86% of ears had stable or improved hearing levels, and all patients maintained preoperative bone conduction thresholds. At a mean follow-up of 48 months, 79% of patients maintained stable or improved pure tone thresholds, whereas 2 subjects experienced delayed sensorineural hearing loss and 2 experienced isolated declining speech discrimination. Notably, 3 of the latter 4 patients were diagnosed with labyrinthine fistula and had undergone radical mastoidectomy. None of the patients who received an intact canal wall tympanomastoidectomy experienced worsening bone conduction thresholds, whereas 1 subject demonstrated a delayed decline in speech discrimination and another recurred. CONCLUSION: It is commonly held that the radical or classic modified radical mastoidectomy is the procedure of choice when managing cholesteatoma in the only hearing ear while intact canal wall techniques are contraindicated. Over the last 20 years, we have adopted a less-rigid, functional approach favoring intact canal wall procedures in the absence of inner ear fistula rather than unequivocally committing to an open cavity. This strategy has been influenced by advancements in preoperative evaluation, increasing familiarity and refinement of closed-cavity techniques, postoperative imaging surveillance options, and the potential for cochlear implant "salvage" in the rare case of profound hearing loss. Based on the current series, this approach appears safe when performed by an experienced surgeon, and reliable long-term patient follow-up is maintained.

Analysis of intersubject variations in intracochlear and middle ear surface anatomy for cochlear implantation.

HYPOTHESIS: We hypothesize that surface landmarks surrounding the round window typically used to guide electrode placement during cochlear implantation (CI) exhibit substantial variability with respect to intracochlear anatomy. BACKGROUND: Recent publications suggest that both atraumatic electrode insertion and electrode location within the scala tympani can affect auditory performance after CI. However, current techniques for electrode insertion rely on surface landmarks alone for navigation, without actual visualization of intracochlear structures other than what can be seen through a surgically created cochleostomy. In this study, we quantify how well the position of intracochlear anatomy is predicted by surface landmarks surrounding the round window. METHODS: Structures representing middle ear surface and intracochlear anatomy were reconstructed in µCT scans of 10 temporal bone specimens. These structures were then reoriented into a normalized coordinate system to facilitate measurement of inter-subject anatomical shape variations. RESULTS: Only minor intersubject variations were detected for intracochlear anatomy (maximum deviation, 0.71 mm; standard deviation, 0.21 mm), with greatest differences existing near the hook and apex. Larger intersubject variations in intracochlear structures were detected when considered relative to surface landmarks surrounding the round window (maximum deviation, 0.83 mm; standard deviation, 0.54 mm). CONCLUSION: The cochlea and its scala exhibit considerable variability in relation to middle ear surface landmarks. While support for more precise, atraumatic CI electrode insertion techniques is growing in the otologic community, landmark guided insertion techniques have limited precision. Refining the CI insertion process may require the development of image-guidance systems for use in otologic surgery.

Cochlear implantation versus hearing amplification in patients with auditory neuropathy spectrum disorder.

OBJECTIVE: Patients with auditory neuropathy spectrum disorder (ANSD) exhibit altered neural synchrony in response to auditory stimuli. Cochlear implantation (CI) is thought to improve neural synchrony in response to auditory stimuli and improve speech perception relative to conventional hearing amplification (HA). STUDY DESIGN: Retrospective review. SETTING: Tertiary otologic practice. SUBJECTS AND METHODS: Subjects included patients with ANSD treated at Vanderbilt University from 1999 to 2011. Sixteen patients underwent CI, and 10 received binaural HAs. Pretreatment performance was assessed through speech reception thresholds and parent questionnaire (Infant-Toddler Meaningful Auditory Integration Scale [IT-MAIS]). Posttreatment outcomes were assessed using IT-MAIS and closed-/open-set speech perception scores. RESULTS: Two HA users underwent neuromaturation and were excluded from further analysis. For the remaining patients, median duration of device use was 48 months. All CI patients had a prior binaural HA trial with failure of auditory skills development. Median available pretreatment IT-MAIS score was 13 and 30 for CI and HA groups, respectively (rank sum test, P = .32). Posttreatment, 6 of 16 CI patients and 4 of 8 HA patients achieved open-set speech perception scores ≥ 60%. No differences between groups were found in posttreatment IT-MAIS scores (rank sum test, P = .11) or the percentage of patients achieving the above levels of open-set speech perception (Fisher exact test, P = .67). CONCLUSIONS: A wide range of speech perception outcomes are observed in ANSD patients. In our ANSD population, patients who exhibited failure of auditory skills development with HAs were able to achieve comparable overall speech perception outcomes after CI relative to those who continued to make appropriate auditory progress with HAs alone.

Outcomes comparing primary pediatric stapedectomy for congenital stapes footplate fixation and juvenile otosclerosis.

OBJECTIVE: To compare presentation, operative findings, and outcomes among pediatric patients undergoing primary stapedectomy for congenital stapes footplate fixation (CSFF) and juvenile otosclerosis (JO). STUDY DESIGN: Retrospective review. SETTING: Combined experience from 2 tertiary academic referral centers. PATIENTS: Pediatric patients with CSFF and JO. INTERVENTION: Primary stapedectomy. MAIN OUTCOME MEASURE(S): 1) Preoperative and postoperative audiometric data using the 1995 AAO-HNS reporting guidelines; 2) Notable operative findings, and postoperative complications. RESULTS: Forty-four pediatric ears met inclusion criteria (27 CSFF, 17 JO). Patients with CSFF presented with a more significant hearing loss (mean PTA 52 dB versus 42 dB; p = 0.04), underwent surgery at a younger age (12.2 versus 16.3 yr; p < 0.001), and more commonly had coincident ossicular malformations (37% versus 0%; p = 0.004). Subjects with JO demonstrated a smaller postoperative ABG (mean 8.8 dB versus 17.2 dB; p = 0.04), although both groups experienced a statistically significant improvement following surgery. Mean bone conduction thresholds remained stable for both groups. There were no instances of profound sensorineural hearing loss, perilymph gusher, facial nerve paresis, or tympanic membrane perforation. CONCLUSION: When performed by an experienced surgeon, stapedectomy is safe and effective in managing carefully selected pediatric patients with CSFF and JO. CSFF is associated with a more severe hearing loss at presentation and concurrent ossicular anomalies are common. Both groups experience substantial benefit from stapedectomy, although ABG closure rates are superior in patients with JO. These data may be helpful in preoperative assessment and patient counseling.

Unilateral auditory performance before and after bilateral sequential cochlear implantation.

OBJECTIVE: The relationship between unilateral preimplantation and postimplantation auditory performance in patients undergoing sequential cochlear implantation (SCI) has not been clearly defined. A greater understanding of this relationship could affect preoperative counseling to patients regarding choice of initial side to implant. STUDY DESIGN: Retrospective case series. SETTING: Tertiary otologic practice. PATIENTS: Adult/pediatric SCI recipients. OUTCOME MEASURES: Unilateral auditory performance preimplantation and postimplantation was assessed. To compare interaural preimplantation performance, we defined a "better-hearing ear" as better pure tone average or speech awareness/reception threshold by at least 10 dB or open/closed-set speech perception score at least 10 percentage points higher. RESULTS: Ninety patients underwent SCI from 1997 to 2011; 34 children and 22 adults with at least 6 months of bilateral implant use underwent further analysis. Preoperatively, the first-implanted ear was better hearing in 6 cases, poorer hearing in 15 cases, and equal hearing in 35 individuals. The proportion of SCI recipients exhibiting better long-term performance of the first-implanted ear was not significantly different from the proportion exhibiting equal or better performance of the second-implanted ear (p = 0.79, χ²), irrespective of preoperative hearing status. The first-implanted ear exhibited better closed/open-set speech perception scores in 41% (9/22) adult and 59% (20/34) pediatric patients at a mean most recent test point of 25 and 39 months, respectively. CONCLUSION: Preimplantation unilateral hearing status was not found to influence relative interaural performance differences after SCI. This finding highlights the relative unimportance of preoperative audiometry and speech recognition scores for guiding clinical decisions regarding implant ear selection.

Stimulation rate reduction and auditory development in poorly performing cochlear implant users with auditory neuropathy.

OBJECTIVE: Patients with auditory neuropathy spectrum disorder (ANSD) exhibit altered neural synchrony in response to auditory stimuli. It has been hypothesized that a slower rate of electrical stimulation in programming strategies for cochlear implant (CI) users with ANSD may enhance development of neural synchrony and speech perception abilities. STUDY DESIGN: Retrospective case series. SETTING: Tertiary otologic practice. PATIENTS: Twenty-two patients with ANSD underwent CI. Patients with complete postoperative audiometric data and at least 2 years of follow-up were included in further analysis. INTERVENTION: Thirteen patients patients met inclusion criteria. Five "poorly performing" CI recipients with ANSD who had not developed closed-set speech perception abilities despite at least 2 years of implant use underwent implant programming to lower the neural stimulation rate. MAIN OUTCOME MEASURES: Speech perception abilities over time using parent questionnaire, closed-set testing, and open-set measures. RESULTS: A high incidence of comorbid conditions was present in the poor performers, including cognitive delay (n = 2), motor delay (n = 3), and autism spectrum disorder (n = 1). The median time to rate slowing in 5 poor performers was 29 months after implant activation. Three of 5 patients achieved closed-set speech perception scores higher than 60% after 6 to 16 months of implant use at the slower rates. At last follow-up (median, 42 mo), no poor performer had yet achieved open-set speech perception abilities. Of all CI recipients with ANSD included in analysis, open-set speech perception abilities developed in 46% (6/13). CONCLUSION: In CI recipients with ANSD who demonstrate limited auditory skills development despite prolonged implant use, lowering the stimulation rate may facilitate acquisition of closed-set speech perception abilities. Further efforts on the study of programming parameters in ANSD patients with CIs are necessary to maximize auditory development in this patient population.