The risk ratio for development of hereditary sensorineural hearing loss in consanguineous marriage offspring.

OBJECTIVES: This study aims to define the relative risk of development of hearing loss in offspring of consanguineous marriages. MATERIALS AND METHODS: This is a retrospective case-control study conducted in a tertiary referral center in Jeddah, KSA. The study group included 1600 probands (848 males, 752 females), with age range 0.5-12 years (6.6 ± 3.6). The study group comprised of two equal, age and sex matched subgroups; Hearing Loss (HL) group and Normal Hearing (NH) group. The children included in the HL group should have idiopathic or non syndromic genetic sensorineural hearing loss. RESULTS: The HL Group comprised 800 children with variable degrees of sensorineural hearing loss. Profound and severe degrees of hearing loss were the most prevalent degrees (P <0.05%). The prevalence of consanguineous marriage offspring in the NH group was 42.5%, while in the HL group it was 68.9% (P < 0.05). The differences between both study subgroups regarding the distribution of different degrees of parental consanguinity (first, second, double first, and first once removed cousins) were insignificant (P > 0.05). The relative risk and 95% confidence interval (RR, 95% CI) for development of hearing loss in offspring of consanguineous marriage was 1.76 (95% CI 1.57-1.97, P < 0.001). CONCLUSIONS: There was 76% increased risk for consanguineous marriage progeny to develop SNHL when compared to non consanguineous progeny.

Clinical criteria of positional vertical nystagmus in vestibular migraine.

OBJECTIVES: The goal of this study was to identify key diagnostic criteria for positional vertical nystagmus caused by vestibular migraine (VM). MATERIALS AND METHODS: The study group included a case series of 13 subjects with VM (10 females and three males with age 38.6 ± 8.9 years); they were complaining of positional vertigo. They were subjected to thorough audiovestibular examination and treated with cinnarizine or topiramate Results: The entire study group demonstrated positional vertical nystagmus (eight patients had up-beating nystagmus and five patients had down-beating nystagmus). The vertigo and nystagmus were elicited in one or more of the following positions: the right & left Dix-Hallpike, supine with head center, head right, and head left positions. The nystagmus had no latency period. It was persistent, non-fatigable and markedly reduced by visual fixation. Prophylactic therapy of VM (topiramate or cinnarizine) cured the positional vertigo in 92% of the study group. Neither the positional vertigo nor the nystagmus recurred in a 3-6 months follow-up period. CONCLUSIONS: VM can induce characteristic form of vertical positional nystagmus and vertigo, which would be treated by medications used for controlling the VM.

Children with chronic lung diseases have cognitive dysfunction as assessed by event-related potential (auditory P300) and Stanford-Binet IQ (SB-IV) test.

Chronic lung disease (CLD) in children represents a heterogeneous group of many clinico-pathological entities with risk of adverse impact of chronic or intermittent hypoxia. So far, few researchers have investigated the cognitive function in these children, and the role of auditory P300 in the assessment of their cognitive function has not been investigated yet. This study was designed to assess the cognitive functions among schoolchildren with different chronic pulmonary diseases using both auditory P300 and Stanford-Binet test. This cross-sectional study included 40 school-aged children who were suffering from chronic chest troubles other than asthma and 30 healthy children of similar age, gender and socioeconomic state as a control group. All subjects were evaluated through clinical examination, radiological evaluation and spirometry. Audiological evaluation included (basic otological examination, pure-tone, speech audiometry and immittancemetry). Cognitive function was assessed by auditory P300 and psychological evaluation using Stanford-Binet test (4th edition). Children with chronic lung diseases had significantly lower anthropometric measures compared to healthy controls. They had statistically significant lower IQ scores and delayed P300 latencies denoting lower cognitive abilities. Cognitive dysfunction correlated to severity of disease. P300 latencies were prolonged among hypoxic patients. Cognitive deficits in children with different chronic lung diseases were best detected using both Stanford-Binet test and auditory P300. P300 is an easy objective tool. P300 is affected early with hypoxia and could alarm subtle cognitive dysfunction.

Vestibular-evoked myogenic potentials and subjective visual vertical testing in patients with vitamin D deficiency/insufficiency.

Otolith function in subjects with vitamin D deficiency/insufficiency is investigated through vestibular-evoked myogenic potentials (VEMP) and subjective visual vertical (SVV) testing. The study group included 62 patients with vitamin D deficiency/insufficiency (30 females, 32 males), with age range 24-56 years (40.6 ± 9.1). The control group included 44 healthy volunteers of similar age and gender distribution. The entire study group had: (1) serum level of 25-hydroxyvitamin D <30 ng/ml; (2) normal bone mineral density as indicated by dual-energy X-ray absorptiometry with T-score >-1; (3) normal middle ear function; (4) Age is ≤60 years. All subjects enrolled in the current study underwent audiovestibular evaluation consisting of pure-tone audiometry, immittancemetry, cervical VEMP (cVEMP), ocular VEMP (oVEMP), and SSV. The entire control group had normal cVEMP, two subjects had abnormal oVEMP. Thirty-three subjects (53%) in the study group had abnormal oVEMP and 31 subjects (50%) had abnormal cVEMP. Forty-one (66%) had abnormal VEMP when abnormal VEMP was considered as either abnormal oVEMP or cVEMP. The entire control and study groups had normal SSV test results. Vitamin D deficiency may be associated with development of otolith dysfunction affecting both the utricle and saccule. This was suggested by the high prevalence of abnormal ocular vestibular-evoked myogenic potentials (oVEMP) and cervical vestibular-evoked myogenic potentials (cVEMP) in the study group.