Should ossicular reconstruction be staged following tympanomastoidectomy.

OBJECTIVES: To determine whether ossicular reconstruction (OCR) performed concurrent with tympanomastoidectomy for cholesteatoma results in significantly different hearing results when compared to OCR performed in a separate, staged procedure. STUDY DESIGN: Retrospective. MATERIALS AND METHODS: Study subjects were patients undergoing OCR within a 2-year period. Intervention was OCR concurrent with tympanomastoidectomy for cholesteatoma (group 1) or staged following tympanomastoidectomy for cholesteatoma (group 2). Main outcome measures were mean postoperative air-bone gap (ABG), proportion achieving ABG closure to <20 dB and <30 dB for group 1 and group 2 overall and when controlling for type of mastoid cavity created (open or closed) and the status of the stapes suprastructure (TORP vs. PORP OCR). RESULTS: Eighty-three patients were identified. Forty underwent OCR concurrent with tympanomastoidectomy and 43 underwent a staged OCR after tympanomastoidectomy. Overall, similar hearing results were seen in the two groups. When considering the status of the stapes and mastoid cavity, concurrent OCR resulted in improved mean postoperative ABG in a closed mastoid cavity with an intact stapes suprastructure (P = .024). Furthermore, a greater proportion of patients who had concurrent OCR within this group achieved ABG <20 dB and ABG <30 dB, although this difference did not reach statistical significance (P = .092 and P = .078, respectively). By contrast, staged OCR resulted in improved mean postoperative ABG and ABG <30 dB in open mastoid cavities with an absent stapes suprastructure (P = .040, and P = .019, respectively). Similarly, a greater proportion achieved ABG <20 dB, approaching statistical significance (P = .055). CONCLUSIONS: Staged OCR is advantageous in those with most severe disease, whereas those with least severe disease may benefit from a concurrent OCR.

Revision BAHA Surgery.

The osseointegrated auditory implant (BAHA) is a system used for hearing rehabilitation through direct bone conduction. Although BAHA surgery is not difficult, the surgeon must observe meticulous technique to prevent complications. Indications for revision BAHA surgery can be divided into (1) failure of fixture osseointegration; (2) bone overgrowth; or (3) skin reaction or skin loss. This article discusses the conditions that might predispose a patient to require BAHA revision, and the steps, if any, that can be taken to prevent these complications. Specific surgical steps for revision of each of these three conditions are also addressed.

Revision stapedectomy.

Revision stapedectomy can be a technically demanding operation. The surgeon must be prepared for many pathologic conditions before revision stapes surgery. Appropriate preoperative patient counseling is a must. The best chance for hearing improvement is in those cases that have a delayed conductive hearing loss after primary stapedectomy. This article serves as a guideline for discussing the myriad possibilities that may be encountered during this type of revision stapedectomy. Indications for revision stapedectomy and general surgical guidelines for management of specific pathologic conditions are discussed. The material presented is based on literature review, the authors' personal experience, and a review of temporal bone studies relative to stapes surgery.

Intratympanic dexamethasone for profound idiopathic sudden sensorineural hearing loss.

OBJECTIVES: To determine hearing recovery by using intratympanic dexamethasone for profound, idiopathic sudden sensorineural hearing loss (ISSNHL). STUDY DESIGN AND SETTING: A prospective, clinical study was performed of 25 consecutive patients seen with profound ISSNHL. Patients received 4 intratympanic treatments of dexamethasone over the course of 2 weeks. Complete hearing recovery was defined as the final pure-tone average (PTA) within 10 dB of baseline. Partial recovery was defined as a final PTA with >/=50% hearing. The hearing in the contralateral ear was used as baseline. RESULTS: The average time to treatment was 28 days. Two patients had significant hearing recovery, and 1 additional patient had partial recovery. The 2 patients with significant hearing recovery were treated within 9 days of onset of hearing loss. CONCLUSIONS: By using the treatment protocol and definition of hearing recovery of this study, intratympanic dexamethasone does not result in significant hearing improvement for patients with profound ISSNHL. There is a possible trend for improved hearing results if intratympanic treatment is performed within 11 days of onset of hearing loss.

Trends in the diagnosis and the management of Meniere's disease: results of a survey.

OBJECTIVE: To determine the practices of the American Neurotology Society (ANS) membership in the evaluation and treatment of the Meniere's patient. STUDY DESIGN: Prospective. INTERVENTION: Questionnaire. MAIN OUTCOME MEASURE: Respondents' response to questions pertaining to the diagnostic and therapeutic practices in the management of Meniere's disease. RESULTS: Three hundred members of ANS were mailed a 15-item questionnaire. Two hundred three responded, for a 67.7% response rate. For the diagnosis of Meniere's disease, 1 in 3 practitioners relied solely on history, physical exam, and audiometry, whereas 2 in 3 relied in part on adjunctive tests, such as electrocochleography (ECOG) and electronystagmography (ENG). Two in 3 practitioners pursued retrocochlear studies on initial evaluation, with the overwhelming majority using MRI. In treating Meniere's disease, conservative medical management was preferred. For medically recalcitrant Meniere's disease, endolymphatic sac surgery (ESS) was the most commonly employed initial intervention (50%), followed by transtympanic gentamicin (38%). Currently, <10% routinely recommend the Meniett device. Eighty-three percent include ESS as a therapeutic option for medically recalcitrant Meniere's disease. The vast majority continue to perform surgical labyrinthectomies and vestibular nerve sections for Meniere's disease. CONCLUSIONS: Meniere's disease continues to pose a difficult diagnostic and therapeutic problem, resulting in heterogeneous approaches to both evaluation and treatment. Despite the 1995 American Academy of Otolaryngology guidelines in the diagnosis of Meniere's disease, most clinicians rely in part on ENG or ECOG in diagnosing Meniere's disease. Furthermore, despite the passing of 20 years since the publications claiming a purely placebo effect, ESS is the most commonly employed initial surgical treatment for Meniere's disease.

Autosomal-dominant Leber Congenital Amaurosis Caused by a Heterozygous CRX Mutation in a Father and Son.

BACKGROUND: Leber congenital amaurosis (LCA) is most often an autosomal recessive disorder. We report a father and son with autosomal dominant LCA due to a mutation in the CRX gene. MATERIALS AND METHODS: DNA screening using an allele specific assay of 90 of the most common LCA-causing variations in the coding sequences of AIPL1, CEP290, CRB1, CRX, GUCY2D, RDH12 and RPE65 was performed on the father. Automated DNA sequencing of his son examining exon 3 of the CRX gene was subsequently performed. RESULTS: Both father and son have a heterozygous single base pair deletion of an adenine at codon 153 in the coding sequence of the CRX gene resulting in a frameshift mutation. CONCLUSION: Mutations involving the CRX gene may demonstrate an autosomal dominant inheritance pattern for LCA.

Audiometric findings of patients with migraine-associated dizziness.

OBJECTIVE: The objective of this study was to determine whether the audiometric findings of migraine-associated dizziness could be used to better distinguish migraine-associated dizziness from Meniere's disease. STUDY DESIGN: A retrospective chart review. SETTING: Tertiary, otology/neurotology practice. PATIENTS: Two groups of patients were studied, a migraine-associated dizziness and a Meniere's disease group. There were 76 and 34 patients in the migraine-associated dizziness and Meniere's disease groups, respectively. INTERVENTIONS: None. MAIN OUTCOME MEASURES: Initial and follow-up pure-tone average and low-frequency pure-tone average were recorded for both groups. Independent samples t tests were used to test for mean differences in pure-tone average and low-frequency pure-tone average. RESULTS: Pure-tone average and low-frequency pure-tone average were significantly worse for patients in the Meniere's disease group at both the initial and follow-up assessments. Three patients in the migraine-associated dizziness group had an elevated pure-tone average (>/=26 dB) and/or low-frequency pure-tone average at initial and/or follow-up assessment. The remaining 73 migraine-associated dizziness patients had normal hearing. In the Meniere's disease group, only two patients had a normal pure-tone average and low-frequency pure-tone average at both initial and follow-up evaluations. The hearing difference between the two groups was significant even when controlling for age and duration of dizziness symptoms. CONCLUSION: Audiometric findings of patients with migraine-associated dizziness are most often normal. Unlike Meniere's disease, the sensorineural hearing loss in migraine-associated dizziness rarely progresses. These audiometric findings may help to distinguish migraine-associated dizziness from Meniere's disease when diagnostic ambiguity exists between these two diagnoses.

Early results with titanium ossicular implants.

OBJECTIVE: To report the efficacy of titanium middle ear prosthesis for ossicular reconstruction. STUDY DESIGN: Retrospective chart reviews were performed for 25 patients who had undergone titanium ossicular implants between January 1, 1999, and June 1, 20001. SETTING: Tertiary otology referral center. PATIENTS: All patients had a minimum of 6 months of postoperative follow-up and no evidence of recurrent otologic disease. INTERVENTION: All patients had undergone ossiculoplasty using titanium middle ear implants. MAIN OUTCOME MEASURES: Comparisons of preoperative and postoperative pure tone averages were performed. Air-bone gap closures and implant extrusion rates were measured. RESULTS Overall mean pure tone averages improved 22.2 dB with air-bone gap improvement at 20.9 dB. Fifty-six percent of patients achieved air-bone gap less than 20 dB postoperatively. The overall extrusion rate was 4%. However, with the placement of cartilage graft interposed between the prosthesis and the tympanic membrane, no extrusion was observed. CONCLUSION: Titanium implants provide comparable hearing improvement compared with other materials. The extrusion rate seems quite low if cartilage interposition graft is inserted. Its ease of handling, biocompatible properties, and sound conducting properties improve its efficacy as an ossicular implant.

Twenty-year review of revision stapedectomy.

OBJECTIVE: To evaluate surgical findings and techniques, patient management techniques, and audiometric results of 522 revision stapedectomies. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary otologic referral center. PATIENTS: A total of 522 revision stapedectomies over a 20-year period in Warren, Ohio, and Israel. The audiologic criterion for revision was an air-bone gap greater than 20 dB over the three-frequency range 0.5 to 2 kHz. RESULTS: Of the 522 revision cases, a total of 483 patients were operated on to improve hearing. The remainder of the patients were operated on for various other noted reasons. Closure of the air-bone gap to within 10 dB was achieved in 71% of patients (343 of 483). The mean pure-tone average improvement was 17.8 dB, with an average postoperative air-bone gap of 7.3 dB. The most common surgical findings were prosthesis malfunction at the oval window, incus, or both (58%). Since beginning the use of the Argon laser for surgical problems, the success rate has increased to 80%. A subgroup of 35 Argon laser revision stapedectomies resulted in a larger hearing gain (25.2 dB) and 91.4% closure of the air-bone gap to less than 10 dB. CONCLUSIONS: More than 70% of revision stapedectomy cases for hearing improvement have had successful closure of their air-bone gap. Since the introduction of the laser 5 years ago, the success rate has increased to 80%. In those specific cases where the laser was required, the success rate increased to 91.4%. Regardless of the revision technique, hearing results were the least successful when the incus could not be used for reconstruction.

Intratympanic steroid treatment: a review.

OBJECTIVE: To review published literature regarding the use of intratympanic steroids in the treatment of Meniere's disease and sudden sensorineural hearing loss and to make recommendations regarding their use based on the literature review. DATA SOURCES: Literature review from 1996 to 2003, PubMed, Medline Plus, and Web of Science. STUDY SELECTION: Retrospective case series and uncontrolled prospective cohort studies were the only types of studies available for review. CONCLUSION: On the basis of the available literature, a weak recommendation is made to use intratympanic steroid treatment of sudden hearing loss if oral steroid therapy fails or is contraindicated. The available studies regarding intratympanic steroid treatment of Meniere's disease and tinnitus are inadequate to answer the question of the efficacy of this treatment for these conditions. Higher quality studies are needed.

Hearing outcomes in stapes surgery: a comparison of fat, fascia, and vein tissue seals.

OBJECTIVE: To compare short- and long-term hearing results following stapedectomy using 3 different oval window grafting materials with the same stapes prosthesis. STUDY DESIGN: Database review. SETTING: Tertiary referral private practice. SUBJECTS AND METHODS: Subjects were ears that underwent stapedectomy for otosclerosis, with placement of fat, fascia, or vein as an oval window seal and reconstruction with a titanium bucket handle prosthesis. A total of 365 procedures met these inclusion criteria: 98 fat grafts, 135 fascia grafts, and 132 vein grafts. Outcome measures included short-term (<1 year) and long-term follow-up air-bone gap. We compared the preoperative and postoperative amount of change in air-bone gap and preoperative and postoperative amount of change in the high-frequency bone conduction average. RESULTS: Overall median times to short-term and long-term follow-ups were 2.2 months and 36.1 months, respectively. There were no statistically significant differences between the 3 tissue seal groups in the amount of change in air-bone gap. There was no significant difference in amount of change in high-frequency bone conduction (representing sensorineural hearing level) between the 3 tissue seal groups. Most patients in all 3 groups had an air-bone gap at long-term follow-up of ≤ 10 dB (fat, 79.5%; fascia, 78.8%; and vein, 75.6%), with 90.3% of all patients at ≤ 20 dB. CONCLUSIONS: In both the short-term postoperative period and long-term follow-up, there were no significant differences in hearing results among 3 types of tissue seals of the oval window in stapes surgery. Fat, fascia, and vein grafts all provide satisfactory hearing outcomes in stapedectomy.

Sound localization in unilateral deafness with the Baha or TransEar device.

OBJECTIVE: To evaluate the sound localization capabilities of patients with unilateral, profound sensorineural hearing loss who had been treated with either a bone-anchored hearing device (Baha BP100) or a TransEar 380-HF bone-conduction hearing device. STUDY DESIGN: Nonrandomized, prospective study. SETTING Tertiary referral private practice. PATIENTS: Patients with unilateral, profound sensorineural hearing loss treated with a BP100 (n = 10) or a TransEar (n = 10) device. Patients wore the hearing device for at least 1 month and had normal hearing in the contralateral ear. Ten patients with normal, bilateral hearing were used for control. INTERVENTIONS: Sound localization of a 3-second recorded sound with and without a TransEar or Baha device was assessed using an array of 7 speakers at head level separated by approximately 45 degrees. The recorded sounds were that of a barking dog or a police siren. Randomized trials of 4 presentations per speaker were given for each hearing condition. MAIN OUTCOME MEASURES: Sound localization was assessed by the accuracy in response and the generalized laterality of response. RESULTS: The mean accuracy of speaker localization was 24% and 26% for the aided condition using the BP100 and TransEar devices, respectively. The mean accuracy of laterality judgment was 59% and 69% for the aided condition using the BP100 and TransEar devices, respectively. These results were only slightly better than chance. There was no statistical difference in localization accuracy or laterality judgment between the BP100 and TransEar groups. CONCLUSION: Neither the BP100 nor the TransEar device improved sound localization accuracy or laterality judgment ability in patients with unilateral, profound sensorineural hearing loss compared with performance in the unaided condition.

Evaluation of a customized acoustical stimulus system in the treatment of chronic tinnitus.

OBJECTIVE: The purpose of this study is to evaluate the efficacy of a customized acoustical stimulus (Neuromonics) system in the treatment of chronic tinnitus. STUDY DESIGN: Multi-institutional prospective. SETTING: Nine U.S. Tertiary Otological referral centers; ambulatory. PATIENTS: Fifty-two adults with chronic tinnitus for a minimum of 6 months, with poor or no response to previous treatments, and not undergoing concomitant therapies were enrolled. INTERVENTIONS: Treatment was delivered in 2 stages: Stage 1 consisted of stimulation with the patient-customized musical tracks and white noise masking of the tinnitus for 2 months, 2 to 4 hours a day. Stage 2 consisted of listening to the same tracks, with no tinnitus masking for 4 months. Both phases included education, cognitive therapy, and periodic follow-up. MAIN OUTCOME MEASURES: Treatment response was measured through validated psychometric testing: the Tinnitus Reaction Questionnaire and the Tinnitus Handicap Inventory. Other measures included the Hospital Anxiety Depression Scale and loudness discomfort levels. RESULTS: Patients' responses were recorded from 38 patients at 6 months, 28 patients at 12 months, and 12 patients at 24 months after initiation of treatment. A total of 14 patients withdrew or were lost to follow-up, and the rest had not reached the 12- or 24-month testing interval. The Tinnitus Reaction Questionnaire score was significantly reduced in 74% of patients at 12 months and 84% of patients at 24 months. Tinnitus Handicap Inventory scores were significantly reduced in 77% of patients at 12 months and 50% at 24 months. CONCLUSION: The customized acoustical stimulus system offers a safe and effective means of tinnitus management for patients with chronic tinnitus.

Gamma knife radiosurgery for vestibular schwannoma.

In 1951, Dr. Lars Leksell from Sweden conceived of what is now known as gamma knife radiosurgery (GKRS). Since Leksell first treated a patient who had a vestibular schwannoma in 1967, there has been a year-to-year increase in the number of patients treated with the gamma knife for vestibular schwannoma. This article outlines the technique of GKRS and discusses the current results of its use to treat vestibular schwannomas. Other topics discussed include tumor control, treatment of recurrent/residual and cystic vestibular schwannomas, and the results of treatment of neurofibromatosis type 2.

Comparison of morning versus evening dosing and 24-h post-dose efficacy of travoprost compared with latanoprost in patients with open-angle glaucoma .

OBJECTIVE: To compare the IOP-lowering efficacy of a.m.-dosed travoprost and latanoprost at 24-h post-dose. RESEARCH DESIGN AND METHODS: Open-angle glaucoma patients not naïve to prostaglandin therapy and currently controlled on p.m.-dosed (2100) latanoprost (n = 21) or travoprost (n = 30) had baseline IOPs measured at 0900. In a randomized, single-masked, crossover design, patients received travoprost (Travatan † ) or latanoprost (Xalatan ‡ ) at 0900 for 4 weeks, then were crossed over to receive the second prostaglandin for another 4 weeks. Treatment IOP was measured at 0900 prior to morning dose at both 4 and 8 week visits. Patient dosing preference (a.m./p.m.) was surveyed on exit. MAIN OUTCOME MEASURE: Intraocular pressure (IOP). † Travatan is a registered trade name of Alcon Laboratories, Inc., Fort Worth, TX, USA ‡ Xalatan is a registered trade name of Pfizer, New York, NY, USA. RESULTS: The mean IOP in the first period when all patients were dosed in the evening was assessed 12 h after dosing at 09:00 and it was similar in the two treatment groups (mean ± standard deviation: 17.9 ± 2.7 mmHg for travoprost versus 17.7 ± 2.5 mmHg for latanoprost, p = 0.812). In the a.m.-dosing crossover comparison, the 24-h post-dose IOP was significantly lower ( p < 0.001) on travoprost (16.9 ± 3.1 mmHg) compared to latanoprost (18.6 ± 3.3 mmHg). In the exit survey, 51% of patients preferred a.m.-dosing. CONCLUSIONS: a.m.-dosed travoprost is superior to a.m.-dosed latanoprost by 1.7 mmHg at 24-h post-dose.

Electrocochleography in patients with Meniere's disease.

OBJECTIVE: The objective of this study was to evaluate the role of electrocochleography (ECoG) in the diagnosis of Meniere's disease. STUDY DESIGN: This study is a retrospective case review. METHODS: Patients undergoing ECoG for Meniere's disease between 1995 and 2003 were identified and segregated as having definite , probable , or possible Meniere's disease according to the 1995 American Academy of Otolaryngology-Head and Neck Surgery guidelines for Meniere's disease classification. Those determined to have probable and possible Meniere's disease were then combined to form a less-than-definite group for statistical analysis. Electrocochleography summating potential (SP)/action potential (AP) ratios were determined, and ratios greater than 0.4 were considered abnormal. The 2 groups were then compared to assess for any correlation between ECoG with the 1995 American Academy of Otolaryngology-Head and Neck Surgery Meniere's disease classification. RESULTS: Sixty patients with definite Meniere's disease and 37 with less-than-definite Meniere's disease were identified. Overall, 59.8% had abnormally elevated SP/AP ratios. Of those with definite Meniere's disease, 66.7% had abnormally elevated SP/AP ratios, whereas of those with less-than-definite Meniere's disease, 52.7% had abnormal ECoGs ( P = .069). CONCLUSIONS: A significant difference in ECoG results was not seen between the definite and less-than-definite Meniere's disease groups. Furthermore, approximately 30% of those with definite Meniere's disease would not be classified as having Meniere's disease based on ECoG results. Because of its lack of sensitivity, ECoG should not play a decisive role in determining the presence or absence of Meniere's disease.