Outcomes of audiometric testing in children with auditory neuropathy spectrum disorder

Association of olfactory neuropathy spectrum disorder and Wolff‐Parkinson‐White syndrome: A Report of a case

“…Olfactory neuropathy spectrum disorder (ONSD) is a rarely seen condition in which congenital hyposmia or anosmia is associated with small or absent olfactory bulbs, analogous to the more frequent auditory neuropathy spectrum disorder (ANSD). 7 ONSD may be present without the hypogonadotropic hypogonadism seen in Kallman syndrome, as was the case with the patient described herein. While a case of hypogonadotropic hypogonadism associated with Wolff-Parkinson-White syndrome (a cardiac condition associated with aberrant myocardial signal conduction that can result in tachycardia, syncope, and fatal dysrhythmias such as supraventricular tachycardia) has been reported, that patient lacked the characteristic anosmia seen in Kallmann syndrome.…”

Auditory Neuropathy Spectrum Disorders: From Diagnosis to Treatment: Literature Review and Case Reports

“…Indeed, hearing loss profiles appear to be variable: from flat to more marked thresholds on low or high frequencies (Figure 3 (a)). ANSD is bilateral in about 75% of cases and unilateral in 25% [134]. The hearing thresholds frequently fluctuate and could reach variations of over 40 dB.…”

“…Fluctuations are more frequent in children than in adults. Some children may show a spontaneous improvement in hearing reactions, more frequently in the first year after diagnosis [134], therefore special attention should be paid to tonal threshold evaluation in infants with ANSD caused by ANSD is bilateral in about 75% of cases and unilateral in 25% [134]. The hearing thresholds frequently fluctuate and could reach variations of over 40 dB.…”

“…Otherwise, a slow hearing deterioration over time is frequently observed in children and adults. Subjects with low frequencies hearing loss frequently show a deterioration overtime in their high and middle frequencies [134,136]. The tonal hearing thresholds are variables within subjects, even when accounted for by the same genetic mutation.…”

“…Impaired speech perception skills are typically out of proportion compared to the tonal thresholds. Background noise further deteriorates residual speech discrimination [1,7,134,137,138].…”