Clinical observations supplemented with imaging examination show that the large vestibular aqueduct syndrome (LVAS) is a rare developmental anomaly of the inner ear, which leads to hearing loss. The authors present a case history, results of imaging examination (high resolution CT, MRI), results of hearing acuity examinations (tonal audiometry, otoacoustic emissions, brainstem auditory evoked p...

Objectives: To determine the frequency and factors associated with inner ear anomalies among cochlear implant candidates. Method: The cross-sectional study was conducted at Department of Otolaryngology, Aga Khan University Hospital, Karachi, from October 2021 to March 2022, comprised patients either gender aged <18 years who received implants. All subjects underwent computed tomography magne...

Preservation of residual hearing after cochlear implantation is now considered an important goal of surgery. However, studies indicate an average post-operative hearing loss of around 20 dB at low frequencies. One factor which may contribute to post-operative hearing loss, but which has received little attention in the literature to date, is the increased stiffness of the round window, due to t...

IMPORTANCE Approximately one-half of all subjects with unilateral or bilateral hearing loss with enlargement of the vestibular aqueduct (EVA) will have SLC26A4 gene mutations. The number (0, 1, or 2) of mutant alleles of SLC26A4 detected in an individual subject with EVA is each associated with a distinct combination of diagnostic and prognostic information as well as probability of recurrence ...

BACKGROUND Nonsyndromic enlargement of vestibular aqueduct (NSEVA) is an autosomal recessive hearing loss disorder that is associated with mutations in SLC26A4. However, not all patients with NSEVA carry biallelic mutations in SLC26A4. A recent study proposed that single mutations in both SLC26A4 and KCNJ10 lead to digenic NSEVA. We examined whether KCNJ10 excert a role in the pathogenesis of N...

Eleven patients with dural fistulas involving the transverse and sigmoid sinuses were treated by transvenous embolization with coils or liquid adhesives. Seven patients underwent preoperative embolization of the external supply followed by direct surgical exposure of the sinus: liquid adhesives were used in four patients and coils in the remaining three. Four of these patients had complete obli...

OBJECTIVE To determine the incidence of high jugular bulb in a group of patients with definite Ménière's disease, and to investigate whether the position or size of the jugular bulb is significantly different in the affected ear than in the unaffected ear. METHODS Retrospective review of patient charts, audiograms, and computed tomography scans to determine the position and size of the jugula...

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