Acquired cholesteatoma after cochlear implants: case series and literature review.

PURPOSE: To assess the prevalence and management of acquired cholesteatoma after cochlear implantation in pediatric and adult patients. METHODS: Retrospective case review of pediatric and adult cochlear implants (CI) followed at a tertiary referral center and literature review of acquired cholesteatoma after CI surgery, to identify its prevalence, cause, and treatment. RESULTS: Nine pediatric CIs were diagnosed with cholesteatoma in seven patients after 6.4 ± 4 years from CI surgery, and two adults after 11.3 and 21.7 years from CI surgery. Thirty-four pediatric cases and 26 adult cases are described in the literature. Cholesteatoma has a prevalence of 0.54% in pediatric CIs, and 1.79% in adult CIs (case series and literature). Adult cases were diagnosed significantly later compared to pediatric cases (Mann-Whitney test, p = 0.0460). Three pediatric cholesteatomas were treated with conservative surgery and preservation of the CI; they all developed recurrent disease. The remaining pediatric cases underwent subtotal petrosectomy with simultaneous CI explantation and staged reimplantation. Only one case recurred. The adult cases underwent simultaneous subtotal petrosectomy, explantation, and reimplantation. Similarly, 33.3% of cases treated with conservative/reconstructive surgery in the literature required revision surgery or conversion to subtotal petrosectomy against 6.2% of subtotal petrosectomies in the literature. CONCLUSIONS: Cholesteatoma after CI is a rare and late-onset complication of CIs. It is more prevalent in the adult CI population, although it affects children significantly earlier. The treatment of choice is subtotal petrosectomy and CI explantation with simultaneous or staged reimplantation.

Management of Vestibular Schwannoma with Normal Hearing.

INTRODUCTION: This work aimed to study the management of vestibular schwannoma (VS) patients with normal hearing (NH). METHODS: A retrospective study was undertaken in a Quaternary referral center for skull base pathologies. Among 4,000 VS patients 162 met our strict audiological criteria for NH. These patients were divided into 2 management groups, wait and scan (W&S) (45/162, 25%) and operated patients (123/162, 75%), and 6 patients were included in both groups. RESULTS: Our management strategy achieved the goals for treatment of VS. First goal, all tumors were completely removed except for 2 intentional residuals. Second goal, facial nerve (FN) function preservation (House Brackmann I, II, and III) was 95.9%. Third goal, possible hearing preservation (HP) attempts occurred in (50/122) (40.9%) with an HP rate in 44% of the patients. Additionally, there were only 2 cases of postoperative complications with no CSF leakage. The prospect of HP in NH patients did not differ with respect to tumor size. However, patients with normal preoperative ABR seemed to have better chances of HP and good FN function and vice versa. HP rate was superior for the MCFA as opposed to the RS + RLA. W&S group demonstrated hearing stability in 88.9% of the patients and FN function stability of HB I in 100% of the patients. CONCLUSIONS: Surgical resection is a reasonable and definitive management option for VS with NH. Nevertheless, choosing to manage cases with observation remains an appropriate management option for NH patients. ABR might be considered as an adjuvant tool indicating better prognosis for HP.

The otologic approach in the management of posterior petrous surface meningiomas.

PURPOSE: Report our experience in the management of posterior petrous surface meningiomas (PPSMs), and identify features that affect hearing, facial nerve (FN) function, and control of the disease. METHODS: Retrospective case series of 131 patients surgically managed for PPSMs. FN status, hearing and tumour radicality were assessed and compared between patients with tumours of different locations (Desgeorges classification) and internal auditory canal involvement (IAC). RESULTS: At the time of surgery 74.8% of patients had a hearing loss. Hearing was mostly unserviceable in tumors attached to the meatus. Pure tone audiometry did not correlate to IAC extension, while speech discrimination scores were statistically worse when the tumor occupied the IAC (unpaired t test, p = 0.0152). Similarly, extrameatal tumors undergoing removal by otic preserving techniques maintained postoperative hearing, whereas hearing worsened significantly in tumors involving the IAC (paired t test, p = 0.048). The FN was affected preoperatively in 11.4% of cases. Postoperative FN palsy was significantly correlated to the IAC involvement (Fisher's exact test, p = 0.0013), while it was not correlated to tumor size. According to the Desgeorges classification, a postoperative FN palsy complicated the majority of anteriorly extending tumors and, two-fifths of meatus centred tumors. 75% of posterior located tumors had a postoperative FN grade I HB. CONCLUSIONS: Since the involvement of the IAC by the tumor affects both hearing and FN function, the IAC is of primary importance in PPSMs and should be studied and addressed as much as the tumor location in the CPA.

The endolymphatic sac tumor: challenges in the eradication of a localized disease.

OBJECTIVE: Identify the critical points that lead to recurrences and lack of radicality in endolymphatic sac tumors (ELSTs). STUDY DESIGN: Retrospective case study and review of the literature. SETTING: Tertiary referral center. PATIENTS: Thirteen cases of ELST were included in the study and their preoperative, intraoperative and postoperative data were analyzed and compared to a review of the literature. INTERVENTION(S): Therapeutical. MAIN OUTCOME MEASURE(S): Prevalence of recurrent and residual tumors, comparison to the literature and analysis of ELST characteristics. RESULTS: Diagnosis was made 26 ± 17 months after the onset of symptomatology, and an ELST was preoperatively suspected in only six cases. At the time of surgery, 10 patients suffered from hearing loss. Preoperative symptoms or audiometry could not predict labyrinth infiltration, although speech discrimination scores were significantly associated with labyrinth infiltration (p = 0.0413). The labyrinth was infiltrated in 8 cases (57.1%), and in 7 cases (46.7%) the tumor eroded the carotid canal, whereas 6 cases (40%) presented an intradural extension. A gross total resection was achieved in 11 cases. There were two residual tumors, one of which because of profuse bleeding, and one recurrence (23.1%). A mean of 22.8% of recurrent or residual tumors are described in the literature based on 242 published cases, in more than half of the cases as a consequence of subtotal tumor resection (STR). CONCLUSIONS: Recurrence derives mostly from the difficulty to identify the extension of the tumor due to the extensive bone infiltration. Accurate diagnosis and correct preoperative planning, with embolization when possible, will facilitate surgery and avoid STR due to intraoperative bleeding. Long follow-ups are important in order to avoid insidious recurrences.

Management of squamous cell carcinoma of the temporal bone: long-term results and factors influencing outcomes.

OBJECTIVE: Temporal bone squamous cell carcinoma (TBSCC) is a rare, aggressive tumor. Surgery, alone or combined with radiotherapy, represent the mainstay of treatment. To report our experience in the treatment of TBSCC and evaluate the disease-specific survival, identifying the factors influencing this outcome. MATERIALS AND METHODS: A retrospective study was performed on 66 patients between 1993 and 2018. Patients were staged according to the University of Pittsburgh-modified TNM staging system. Nine cases (13.6%) were Stage I, 7 cases (10.6%) Stage II, 20 cases (30.3%) Stage III and 30 cases (45.5%) Stage IV. Twenty-four patients underwent lateral temporal bone resection (LTBR) and 42 patients underwent subtotal temporal bone resection (STBR). RESULTS: One hundred percent of Stage I and II patients showed no evidence of disease (NED) after a median follow-up of 101 months (range 1-289 months). NED resulted in 88.2% of Stage III (mean follow-up 80.3 months; range 8-257) and 46.4% of stage IV (mean follow-up 50.6 months; range 3-217). Pittsburgh Stage or involvement of mastoid, facial nerve, medial wall of the middle ear, temporomandibular joint and middle fossa dura emerged as negative prognostic factors. The highest mortality rate occurred in the first 2 years after treatment, due to local recurrence. CONCLUSIONS: Prognosis of TBSCC can be excellent in early stage tumors, employing a LTBR. In more advanced cases, prognosis is poor. STBR with adjuvant radiotherapy represents the treatment of choice, offering acceptable survival rates. Given the rarity of the pathology, many controversies still exist concerning optimal management.

A New Treatment Option in Incomplete Partition Type III: The Varese Bone-Air Stimulation (B.A.S.).

The incomplete partition type III is a severe cochlear malformation present in X-linked deafness. It is a rare, non-syndromic cause of severe to profound mixed hearing loss, often progressive. The complete absence of bony modiolus and the wide communication between the cochlea and the internal auditory canal make cochlear implantation challenging, with still no consensus on the management of these patients. To the best of our knowledge, no results have ever been published in the literature on the treatment of these patients with hybrid stimulation (bone and air). We present three cases in which this hybrid stimulation gave better audiological results then air stimulation alone. A literature review on audiological results of the current treatment options in children affected by IPIII malformation was conducted independently by two researchers. Ethical considerations on the treatment of these patients were conducted by the Bioethics department of the University of Insubria. In two of the patients, the bone-air stimulation, associated with prosthetic-cognitive rehabilitation, meant that surgery was avoided, obtaining similar communication performances of those present in the literature. We believe that, when the bone threshold appears partially preserved, a stimulation through the bone or hybrid modality, such as the Varese B.A.S. stimulation, should be attempted.

Meningiomas of the Internal Auditory Canal.

OBJECTIVE: Identify and define specific preoperative and postoperative characteristics of intracanalicular meningiomas (ICMs) in order to improve their diagnosis and management, and to differentiate them from intrameatal vestibular schwannomas (IMVSs). METHODS: Preoperative symptomatology, magnetic resonance imaging (MRI), and postoperative outcomes of 28 ICMs were analyzed. The results were compared to the literature and IMVSs treated by our group. RESULTS: Anacusis and progressive hearing loss were more frequent in the present population than the cases reviewed (P = .0064 and P = .0001, respectively). Hearing loss affected more than 90% of the patients, with anacusis in 32.1% of the cases. Facial palsy affected 17.9% of the patients. In comparison to IMVSs, preoperative anacusis was more associated to meningiomas (P = .0037), and the facial nerve was more compromised in ICMs than IMVSs, both preoperatively (P = .0011) and at follow-up (P < .0001). According to a re-evaluation of preoperative MRIs and comparison with IMVSs, linear tumor borders, and linear morphology along the internal auditory canal wall, but not the presence of a dural tail, were significantly more present in ICMs (P = .0035, P = .0004, P = .1963, respectively). These characteristics could have led to a correct preoperative diagnosis in 61% of our cases. CONCLUSION: Contrariwise to IMVSs, the frequent preoperative anacusis and facial palsy demonstrate the more aggressive nature of ICMs, which also carry a higher risk of postoperative facial palsy and difficulty to preserve hearing. An attentive evaluation of imaging should ease diagnosis, and asymptomatic or stable ICMs should be enrolled in a wait-and-scan protocol. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E413-E419, 2021.

Long term outcomes of canal wall up and canal wall down tympanomastoidectomies in pediatric cholesteatoma.

Cholesteatomas in children have a more aggressive growth pattern compared to adults, which leads to a higher incidence of both residual and recurrent disease. A staged canal wall-up or a canal wall-down tympanomastoidectomy (CWUT and CWDT, respectively) is selected depending on the extent of the disease and condition of the middle ear (ME) cleft and mastoid. Endoscopic ear surgery (EES) has been recently introduced as an adjuvant tool for the treatment of this pathology even in the pediatric population. OBJECTIVES: To analyze long term outcomes of CWUT and CWDT in the pediatric population, focusing on residual and recurrence rates of cholesteatoma and hearing results. A literature review including cases treated with EES were discussed. MATERIAL AND METHODS: Pediatric patients treated for cholesteatoma involving both the ME and mastoid with a follow-up (FU) of at least 4 years were retrospectively analyzed in a quaternary referral center for otology and lateral skull base surgery. Patients were grouped according to the surgical technique (CWUT versus CWDT). Rates of residual and recurrent cholesteatoma after each surgical technique were reported and compared. Mean Air-Bone Gap (ABG) of 0.5-1-2-4 KHz was measured and reported before the first surgery and at the last post-operative FU. RESULTS: Two-hundred and thirty-six cases fulfilled our inclusion criteria. The mean FU was 100.4 ± 44.2 months (median 89 months). One-hundred and five (44.5%) cases underwent a CWUT, whereas 131 (55.5%) a CWDT. A second stage surgery was performed in 73.5% of CWUT and 58.7% of CWDT. Among the CWUT group, residual cholesteatoma occurred in 22 (21%) ears and recurrence in 24 (22.9%). Patients undergoing CWDT showed lower rates of both residual and recurrent cholesteatoma (7.6% and 2.3%, respectively). ABG improvement was noted for both groups, even though CWUT showed better post-operative hearing results. CONCLUSIONS: The CWDT technique offers a definite surgical therapy, with minimal residual and recurrence rates and audiological results comparable to the CWUT technique. EES must still prove its added benefit or equivalence to pure microscopic approaches.

HIC1 Represses Atoh1 Transcription and Hair Cell Differentiation in the Cochlea.

Across species, expression of the basic helix-loop-helix transcription factor ATOH1 promotes differentiation of cochlear supporting cells to sensory hair cells required for hearing. In mammals, this process is limited to development, whereas nonmammalian vertebrates can also regenerate hair cells after injury. The mechanistic basis for this difference is not fully understood. Hypermethylated in cancer 1 (HIC1) is a transcriptional repressor known to inhibit Atoh1 in the cerebellum. We therefore investigated its potential role in cochlear hair cell differentiation. We find that Hic1 is expressed throughout the postnatal murine cochlear sensory epithelium. In cochlear organoids, Hic1 knockdown induces Atoh1 expression and promotes hair cell differentiation, while Hic1 overexpression hinders differentiation. Wild-type HIC1, but not the DNA-binding mutant C521S, suppresses activity of the Atoh1 autoregulatory enhancer and blocks its responsiveness to ß-catenin activation. Our findings reveal the importance of HIC1 repression of Atoh1 in the cochlea, which may be targeted to promote hair cell regeneration.

Surgical Labyrinthectomy and Cochlear Implantation in Menière's Disease.

OBJECTIVE: The aim of this study was to analyze the results of labyrinthectomy and cochlear implantation (CI) on hearing, vertigo, and tinnitus and evaluate the adequacy of labyrinthectomy and CI for the treatment of end stage Menière's Disease (MD). STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Charts of 22 patients undergoing labyrinthectomy and CI in the same ear for intractable vertigo and hearing loss with both preoperatory and postoperatory documentation available, were reviewed. INTERVENTION(S): Therapeutic. MAIN OUTCOME MEASURE(S): Auditory outcomes were assessed with pure tone and speech audiometry, and compared with the preoperatory audiometric evaluation. Dizziness was graded according to the Dizziness Handicap Inventory Questionnaire (DHI). Tinnitus outcomes were assessed by the tinnitus handicap inventory (THI). RESULTS: Post-CI pure tone average had a statistically significant improvement (p = 0.035, paired t test). Speech audiometry resulted in a non-statistically significant speech discrimination score variation (p = 0.056, paired t test). Postoperatory THI had a statistically significant variation (p = 0.0001, paired t test). Sixty seven per cent of the patients had complete resolution of the vestibular symptoms in their operated ear, however, patients over 70 years old had significantly more failures as evinced by the postoperative DHI (p = 0.0109, Fisher's exact test). CONCLUSIONS: Patients affected by end stage MD or secondary MD, with vertigo and severe hearing loss can successfully undergo labyrinthectomy and CI. Caution should be reserved in elderly patients for a risk of persistent instability. The CI confers significant benefit in hearing rehabilitation and tinnitus suppression.

Management of a cocaine-induced palatal perforation with a nasal septal button.

A cocaine-induced midline destructive lesion (CIMDL) is a rare consequence of cocaine insufflation that involves the nose, sinuses, and occasionally the palate. Palatal perforations compromise swallowing, mastication, and speech. An obturator prosthesis can be used to overcome these complications. In selected cases, a nasal septal button is a good alternative for the sealing of a palatal perforation, especially when surgery is not indicated, such as in cases of persistent cocaine abuse. Abstinence from cocaine is the most effective long-term management option for patients with a CIMDL, and surgical correction of the defect should be postponed until the patient stops sniffing cocaine and the lesion becomes stable. We describe the case of a 39-year-old cocaine abuser whose oronasal communication was plugged with a nasal septal button, which resulted in an immediate alleviation of his oronasal reflux.