The association between consanguineous marriage and offspring with congenital hearing loss.

BACKGROUND: Consanguinity is a commonly recognized practice among marriages in the Middle East and may lead to an increase in the prevalence of inherited disorders. Autosomal recessive deafness is the most common form of inherited congenital hearing loss (CHL). OBJECTIVES: Determine the association of consanguineous marriages with congenital sensorineural hearing loss (SNHL) and auditory neuropathy. DESIGN: Descriptive and analytical cross-sectional study. SETTING: Ear specialist hospital. PATIENTS AND METHODS: Children with severe-to-profound congenital SNHL, who had been referred to the specialist hospital for cochlear implant were analyzed. Patients were divided into subgroups based on degree of consanguinity. MAIN OUTCOME MEASURE: The relative risk of having more than one child with SNHL in offspring of a consanguineous marriage. SAMPLE SIZE: 189 parents and children with CHL. RESULTS: The parents of 157 children (83.1%) were blood-related. Of those, 48 had more than one child with CHL (31.4%), while only two parents who were not blood-related had more than one child with CHL (6.25%; P=.005). Among the 189 children, 131 (69.3%) parents were direct cousins. Only 39 (20.6%) and 43 (22.8%) children had family histories of CHL on the paternal and maternal sides, respectively. There was no statistically significant difference in the prevalence of auditory neuropathy between the offspring of consanguineous and non-consanguineous marriages (P=.648). CONCLUSION: The risk of having more than one child with SNHL in the offspring from a consanguineous marriage is 3.5 times higher than that of a non-consanguineous mating. LIMITATION: The association of hearing loss degree with consanguinity was not studied. CONFLICT OF INTEREST: None.

The acute auditory effects of exposure for 60 minutes to mobile`s electromagnetic field.

OBJECTIVE: To assess the immediate consequences of 60 minutes exposure to mobile phones on hearing function by determining changes in distortion product otoacoustic emission (DPOAE) and hearing threshold levels (HTLs). METHODS: This prospective control clinical trial study was carried out at the Ear, Nose and Throat Department, King Abdulaziz University Hospital, Riyadh, Kingdom of Saudi Arabia from July 2009 to July 2011. The data collected included age, symptoms experienced after exposure, and HTLs and DPOAE were recorded before, and immediately after 60 minutes of exposure to the same model of mobile phone. RESULTS: Heat/pain was the most commonly reported symptom. In the test-ears, significant shift (p<0.05) was noticed in HTLs at 1000 and 2000 Hz but not at other frequencies, while non test-ears did not reveal significant shift in HTLs. Additionally, test-ears revealed significant differences (p<0.05) in DPOAE at 1000 Hz, 1400 Hz, 2000 Hz, and at the average of all frequencies, while non test-ears did not show significant differences. CONCLUSION: Sixty minutes of close exposure to electromagnetic fields emitted by a mobile phone had an immediate effect on HTL assessed by pure-tone audiogram and inner ear (assessed by DPOAE) in young human subjects. It also caused a number of other otologic symptoms.

A preliminary study of endoscopic acoustic stapedial reflex in chronic otitis media.

OBJECTIVE: To introduce the endoscopic acoustic stapedial reflex (EASR) as a technique for assessing stapedial mobility in ears with chronic otitis media (COM). METHODS: This prospective study was performed from February 2008 to February 2009, patients with COM presented to the Otology Clinic, King Abdul-Aziz University Hospital, Riyadh, Kingdom of Saudi Arabia were examined with a rigid ear endoscope to visualize the stapedius tendon and the head of stapes. The reflex is elicited by pulsed sound stimuli. The stapedius muscle contraction and the mobility of the stapes are visualized during exposure ipsilaterally, and contralaterally to sound stimuli. RESULTS: Conventional tympanometry did not detect a stapedial reflex in any of the 10 patients. Acoustic stapedial reflex was detected in all ears. Despite a visible stapedial muscle contraction, one patient did not demonstrate stapes movement, and was confirmed to have stapes fixation at surgery. All patients tolerated the test with no complications, or side effects. CONCLUSION: The EASR was introduced for the first time. In addition to the well-known value of the stapedial reflex testing, it provides a safe, and reliable method for assessment of stapes mobility.

Hearing Improvement after Partial Labyrinthectomy: Resection of petrous apex cholesterol granuloma.

We present the case of a 57 year-old male presenting with symptomatic petrous apex cholesterol granuloma, multiple cranial nerve weaknesses and deafness of the left ear. The chosen intervention was a cholesterol granuloma resection via the translabyrinthine approach. This resulted in cranial nerve recovery and improved facial functionality. Hearing in the operated ear improved within 3 months after surgery. The patient experienced no postoperative complications. We conclude that a translabyrinthine approach can be used for drainage of petrous apex cholesterol granulomas with a chance of hearing loss recovery.

High-resolution X-ray computed tomographic scanning of the human stapes footplate.

OBJECTIVES: The mechanical behaviour of the footplate and its annular ligament depends critically on their shape and orientation in the oval window, but accurate measurements have been difficult to make owing to their small size. Our aims are to visualize the footplate at high resolution and understand its dynamics. METHODS: The human cadaver stapes footplate was dissected, and very high-resolution x-ray computed tomographic (CT) scans, with voxel sizes from 4 to 8 pm, were performed. Locally developed software was used to reconstruct the images. RESULTS: The data permit us to explore minor details of shape and orientation. The footplate looks like a footprint, and the annular ligament has variable thickness, with a cleft (groove) in its anterior attachment to the oval window. The CT data also permit us to create a three-dimensional finite-element model that can simulate footplate motion. CONCLUSIONS: The results obtained lead to further understanding of the mechanical behaviour of the footplate and the annular ligament.