Barbara S. Herrmann

Superior semicircular canal dehiscence presenting as conductive hearing loss without vertigo.

Objective: The objective of this study was to describe superior semicircular canal dehiscence (SSCD) presenting as otherwise unexplained conductive hearing loss without vestibular symptoms. Study Design: Retrospective. Setting: Tertiary referral center. Patients: The study comprised 8 patients (10 ears), 5 males and 5 females aged 27 to 59 years. All 10 ears had SSCD on high-resolution computed tomography scan of the temporal bone. Diagnostic Tests and Results: All 10 ears had significant conductive hearing loss. The air–bone gaps were largest in the lower frequencies at 250, 500, and 1000 Hz; the mean gaps for these 3 frequencies for the 10 ears were 49, 37, and 35 dB, respectively. Bone-conduction thresholds below 2000 Hz were negative (−5 dB to −15 dB) at one or more frequencies in 8 of the 10 ears. There were no middle ear abnormalities to explain the air–bone gaps in these 10 ears. Computed tomography scan and laboratory testing indicated lack of middle ear pathology; acoustic reflexes were present, vestibular evoked myogenic potentials (VEMPs) were present with abnormally low thresholds, and umbo velocity measured by laser Doppler vibrometry was above mean normal. Middle ear exploration was negative in six ears; of these six, stapedectomy had been performed in three ears and ossiculoplasty in two ears, but the air–bone gap was unchanged postoperatively. The data are consistent with the hypothesis that the SSCD introduced a third mobile window into the inner ear, which in turn produced the conductive hearing loss by 1) shunting air-conducted sound away from the cochlea, thus elevating air-conduction thresholds; and 2) increasing the difference in impedance between the oval and round windows, thus improving thresholds for bone-conducted sound. Conclusion: SSCD can present with a conductive hearing loss that mimics otosclerosis and could explain some cases of persistent conductive hearing loss after uneventful stapedectomy. Audiometric testing with attention to absolute bone-conduction thresholds, acoustic reflex testing, VEMP testing, laser vibrometry of the umbo, and computed tomograph scanning can help to identify patients with SSCD presenting with conductive hearing loss without vertigo.

Vestibular evoked myogenic potentials show altered tuning in patients with Ménière's disease.

Objective: Acoustic stimulation of the saccule gives rise to a vestibulocollic reflex, the output of which can be measured in the neck as inhibition of activity in the ipsilateral sternocleidomastoid muscle. This vestibular evoked myogenic potential has been promoted as a means of assessing integrity of saccular function. In this study, we test the hypothesis that the cochleosaccular hydrops of Ménière’s syndrome leads to alterations in saccular motion that change the dynamics of the vestibular evoked myogenic potential. Study Design: Prospective cohort study. Setting: Large specialty hospital, department of otolaryngology. Subjects: Fourteen normal adult volunteers and 34 consecutive consenting adult patients with unilateral Ménière’s disease by American Academy of Otolaryngology–Head and Neck Surgery diagnostic criteria. Interventions: All subjects underwent vestibular evoked myogenic potential testing using ipsilateral broadband click and short tone-burst stimuli at 250, 500, 1,000, 2,000, and 4,000 Hz. Main Outcome Measures: Threshold, amplitude, and latency of vestibular evoked myogenic potential responses in normal and Ménière’s affected and unaffected ears. Results: Vestibular evoked myogenic potential was present in all ears tested. Normal subjects show a frequency-dependent vestibular evoked myogenic potential threshold, with best response (“frequency tuning”) at 500 Hz. Compared with normal subjects and unaffected ears of Ménière’s subjects, affected Ménière’s ears had significantly increased vestibular evoked myogenic potential thresholds. Affected Ménière’s ears showed threshold shifts at all frequencies and there was less tuning apparent at 500 Hz. Unaffected ears of Ménière’s subjects also showed significantly elevated vestibular evoked myogenic potential thresholds compared with normal subjects. Analyses of vestibular evoked myogenic potential thresholds for effects of age, hearing loss, and audiometric configuration showed no significant differences. Conclusions: Ménière’s ears display alterations in vestibular evoked myogenic potential threshold and tuning, supporting our hypothesis of altered saccular motion mechanics arising from hydropic distention. Unaffected ears of unilateral Ménière’s subjects show similar changes, though to a lesser degree. This finding may be because of occult saccular hydrops in the asymptomatic ear or binaural interactions in the vestibular evoked myogenic potential otolith–cervical reflex arc.

Vestibular evoked myogenic potentials (VEMP) can detect asymptomatic saccular hydrops.

Objective: The objective of this study was to explore the useful of vestibular evoked myogenic potential (VEMP) testing for detecting endolymphatic hydrops, especially in the second ear of patients with unilateral Ménière disease (MD).

Vestibular evoked myogenic potential (VEMP) in patients with Ménière's disease with drop attacks.

Objective: In this retrospective study, we tested the hypothesis that vestibular evoked myogenic potential (VEMP) thresholds are more often elevated or absent in patients with Ménières disease experiencing Tumarkin drop attacks than in other patients with Ménières disease.

Vestibular evoked myogenic potentials versus vestibular test battery in patients with Meniere's disease.

Objective: The present study was undertaken to assess the sensitivity of vestibular evoked myogenic potentials testing to side-of-disease in Ménière’s disease patients and to test the hypothesis that information supplied by vestibular evoked myogenic potentials is complementary to that provided by a conventional vestibular test battery. Study Design: Prospective cohort study. Setting: Large specialty hospital, department of otolaryngology. Subjects: Twenty consenting adults (9 men and 11 women) with unilateral Ménière’s disease by American Academy of Otolaryngology–Head and Neck Surgery diagnostic criteria. Interventions: All subjects underwent bilateral vestibular evoked myogenic potentials testing using ipsilateral broadband click and short-toneburst stimuli at 250, 500, and 1,000 Hz. All subjects also underwent electronystagmography and sinusoidal vertical axis rotation testing. Main Outcome Measures: Accuracy of side-of-disease assignment by vestibular evoked myogenic potentials, caloric asymmetry, and multivariate analysis. Results: Side-of-disease assignment was most accurate using caloric asymmetry with a 5% interaural difference criterion, achieving 85% correct assignment. The next best method was vestibular evoked myogenic potentials using 250-Hz toneburst stimuli, achieving 80% correct assignment. The least accurate method was caloric asymmetry using a traditional 30% interaural difference limen, achieving 55% correct assignment. Comparison of 5% interaural difference criterion and vestibular evoked myogenic potentials using 250-Hz toneburst stimuli showed discordant results, but in no case did both 5% interaural difference criterion and vestibular evoked myogenic potentials using 250-Hz toneburst stimuli make an incorrect assignment. Conclusion: Vestibular evoked myogenic potentials threshold was shown to be highly sensitive to side-of-disease in unilateral Ménière’s disease. We observed instances of discordance in side-of-disease assignment by caloric asymmetry and vestibular evoked myogenic potential methods but no case in which both methods were incorrect. This supports the hypothesis that vestibular evoked myogenic potentials supplies information complementary to that provided by other components of the vestibular test battery.

Nucleus 22 Cochlear Implantation Results in Postmeningitic Deafness

Cochlear implant surgery was performed on 13 patients with postmeningitic deafness (seven adults, six children). Two adults and two children (30.8%) had severe labyrinthitis ossificans requiring radical “drill‐out.” Five of 13 (38.5%) had some bone growth requiring partial drill‐out, and four of 13 (30.8%) had normal insertion with no drill‐out. Hearing results for patients with no bone growth were similar to nonmeningitic patients; three of four (75%) had open‐set speech recognition. Performance of patients with total drill‐out was poor; “auditory only” performance was limited to detection and pattern perception of speech, and no patients had open‐set speech recognition. Results for patients with partial drill‐out were similar to results in patients with no bone growth. Labyrinthitis ossificans not only presents surgical challenges to cochlear implantation but may also adversely affect hearing outcome.

Differential cochlear implant outcomes in older adults

The goals of this study were to analyze whether cochlear implant (CI) users over 65 years of age have different surgical and audiological outcomes when compared to younger adult CI users and to identify reasons for these possible differences.

Cochlear implantation in children with anomalous cochleovestibular anatomy: a systematic review.

Objective. To determine the influence of inner ear dysplasia on both surgical and audiologic outcomes following pediatric cochlear implant (CI) surgery. Data Sources. MEDLINE (1982-2009) and data from Massachusetts Eye and Ear Infirmary. Review Methods. A systematic review of the literature was performed. Variables assessed included age at implantation, duration of CI use, radiologic and operative findings, and speech perception outcome data. Results. The initial search yielded 1326 articles. Including data from our own study, twenty-two fulfilled criteria for inclusion, representing 311 patients. Data for bilateral implants were recorded only for the first implant. Data on simultaneous bilateral implants were not recorded. The most common anomaly seen was large vestibular aqueduct (89/311 or 29%). When comparing patients with mild-moderate or severe dysplasia, rates of cerebrospinal fluid gusher were 31% versus 35% (odds ratio [OR] = 0.50), anomalous facial nerve anatomy was seen in 11% versus 51% (OR = 0.15), and postoperative speech perception abilities were found in 84% versus 54% (OR = 1.93), respectively. A large heterogeneity was found among studies regarding all outcome measures. Conclusion. Although we found that severe inner ear dysplasia was associated with increased surgical difficulty and lower speech perception, the lack of uniformity in published clinical data limited the strength of these results. Standardization of surgical and radiologic reporting as well as more consistent speech perception testing is needed to better determine the association between anomalous cochleovestibular anatomy and clinical outcomes.

Cochlear implantation in children with congenital X-linked deafness due to novel mutations in POU3F4 gene.

Objectives: We report novel mutations in the POU3F4 gene resulting in congenital X-linked deafness DFN3, and describe the results of cochlear implantation in 4 boys (3 siblings) followed for an average of 3.5 years. Methods: The diagnosis of DFN3 was made in infant boys on the basis of the radiologic criteria of an underdeveloped modiolus, a wide cochlear fossette, and the presence of all cochlear turns. The POU3F4 gene was sequenced. A standard, transmastoid, facial recess approach was used for cochlear implantation. A lumbar drain was placed before the operation. Results: The identified mutations in the POU3F4 gene were novel (p.R167X in the 3 siblings) or recently reported (p.S310del). A high-flow cerebrospinal fluid leak through the cochleostomy was encountered in each patient and was ultimately controlled. Although the implants functioned properly, the auditory perceptual abilities did not progress past sound detection in the 3 siblings, or past closed-set word identification in the non-sibling, who achieved better speech perception with contralateral amplification. Three boys (2 siblings) show signs of other learning disorders; 1 boy was too young for a complete assessment. Conclusions: Preoperative gene mutation analysis in DFN3 patients who are considering cochlear implantation may help in long-term counseling and in avoidance of postoperative complications. Limited auditory perception and language acquisition may result. Amplification may sometimes be a better alternative than cochlear implantation, despite the severity of the hearing loss.

Failure to Clinically Predict NICU Hearing Loss

Neonatal intensive care unit (NICU) survivors demonstrate handicapping sensorineural hearing loss up to 50 times more frequently than normal newborns, yet little is known about the etiology of the hearing loss. Theoretically, accurate identification and triage of a particular infant based on a clinical profile would be useful. Forty NICU graduates of The Massachusetts General Hospital were selected for a detailed retrospective chart review evaluating prenatal, perinatal, and NICU medical conditions and treatment. Twenty-three patients identified with hearing loss and 17 infants with normal hearing were compared clinically. Univariate and multivariate analysis was performed on a subpopulation of patients (20 with hearing loss and 16 with normal hearing). A history of ventilation was associated with hearing loss (P=.0023), but this factor was not absolute. No other clinical parameters were convincingly linked to hearing loss. We conclude that reliance on risk factors is an inadequate clinical method to select a patient for a hearing test and that each NICU survivor deserves audiometric evaluation.