Characterization of sensorineural hearing loss in children with Alport syndrome

Most adults with Alport syndrome (AS) suffer from progressive sensorineural hearing loss. However, little is known about the early characteristics of hearing loss in children with AS. As a part of the EARLY PRO-TECT Alport trial, this study was the first clinical trial ever to investigate hearing loss in children with AS over a timespan of up to six years Nine of 51 children (18%) had hearing impairment. Audiograms were divided into three age groups: in the 5–9-year-olds, the 4-pure tone average (4PTA) was 8.9 decibel (dB) (n = 15) in those with normal hearing and 43.8 dB (n = 2, 12%) in those with hearing impairment. Among the 10–13-year-olds, 4PTA was 4.8 dB (healthy, n = 12) and 41.4 dB (hearing impaired, n = 6.33%). For the 14–20-year-olds, the 4PTA was 7.0 dB (healthy; n = 9) and 48.2 dB (hearing impaired, n = 3.25%). On average, hearing thresholds of the hearing impaired group increased, especially at frequencies between 1–3 kHz. In conclusion, 18% of children developed hearing loss, with a maximum hearing loss in the audiograms at 1–3 kHz. The percentage of children with hearing impairment increased from 10% at baseline to 18% at end of trial as did the severity of hearing loss.

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Alport Syndrome: The Eye as a Window to the Human Body

10.26715/jbms.33_2021_3_8 ◽

2021 ◽

Vol 33 (3) ◽

pp. 46-48

Author(s):

Aysha Tareq Nusef ◽

Abdulla Almoosa ◽

Wael Wagih Aly

Keyword(s):

Hearing Loss ◽

Case Report ◽

Renal Insufficiency ◽

Sensorineural Hearing Loss ◽

Alport Syndrome ◽

Crystalline Lens ◽

Secondary Hypertension ◽

Sensorineural Hearing ◽

Blurred Vision ◽

Ophthalmic Manifestations

Alport syndrome (AS) is a rare genetic disease affecting type four collagen production, causing renal, auditory, and ophthalmic manifestations. This case report is about a 32-year-old male who was a known case of renal insufficiency and secondary hypertension and was referred to the ophthalmology department due to blurred vision. Based on the patient‘s history and ophthalmological findings, AS was diagnosed. Ophthalmic examination showed anterior lenticonus associated with sensorineural hearing loss (SNHL) and impaired renal function. This clinical case report sheds light on the role of ophthalmology in diagnosing AS. Keywords: Collagen, Crystalline lens, Hereditary nephritis, Ophthalmology, Renal insufficiency, Sensorineural hearing loss

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Audiometric Characterization of a Family with Digenic Autosomal, Dominant, Progressive Sensorineural Hearing Loss

CrossRef Listing of Deleted DOIs ◽

10.1080/000164800760370837 ◽

2000 ◽

Vol 120 (1) ◽

pp. 51-57 ◽

Cited By ~ 3

Author(s):

Erik Borg ◽

Eva Samuelsson ◽

N. Dahl

Keyword(s):

Hearing Loss ◽

Sensorineural Hearing Loss ◽

Autosomal Dominant ◽

Sensorineural Hearing

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Alport syndrome

Nepalese Journal of Ophthalmology ◽

10.3126/nepjoph.v1i2.3690 ◽

1970 ◽

Vol 1 (2) ◽

pp. 139-140 ◽

Cited By ~ 2

Author(s):

P Karki ◽

JK Shrestha

Keyword(s):

Hearing Loss ◽

Sensorineural Hearing Loss ◽

Male Patient ◽

Alport Syndrome ◽

Young Male ◽

Clinical Findings ◽

Sensorineural Hearing ◽

Ocular Findings

Alport syndrome is an oculo-renal syndrome characterized by a triad of clinical findings consisting of hemorrhagic nephritis, sensorineural hearing loss and characteristic ocular findings. We report a young male patient who presented with painless diminution of vision associated with hearing loss. The importance of ophthalmic evaluation for suspecting the disease is highlighted. Keywords: Alport syndrome; sensorineural hearing loss; micro-hematuria; oculo-renal syndrome DOI: 10.3126/nepjoph.v1i2.3690 Nep J Oph 2009;1(2):139-140

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