Positive family history of idiopathic sudden sensorineural hearing loss

Abstract

Idiopathic sudden sensorineural hearing loss (ISSNHL) is a heterogenic disease. Multiple factors influencing aetiology and prognosis are discussed. A retrospective clinical characterisation and analysis of family history of ISSNHL patients was performed to investigate influences on the disease. 186 inpatients diagnosed with ISSNHL were characterised by health records and a standardised questionnaire. Audiograms were observed. 75 controls that had never experienced an event of ISSNHL were questioned about family members being affected by ISSNHL. 63.4% of all patients could be assigned to at least one group with similar causes of ISSNHL (noise exposure, positive family history, infectious diseases, hypothyroidism and fibromuscular dysplasia). A positive family history for ISSNHL has not been reported so far. Therefore, we accentuated the characterisation of patients with positive family history. 21.4% affirmed a positive family history. In ten families, at least two family members were reported as ISSNHL patients. In comparison with patients with negative family history, they tend to be younger, experience more events of ISSNHL and show less improvement of hearing abilities under therapeutic treatment (non-significant). Differences intensified between smokers with positive family history and non-smokers with negative family history. Differences concerning average age were statistically significant (p = 0.001). Within 75 controls 11 families were reported with one member being affected by ISSNHL. In the control group we did not detect any family with more than one ISSNHL patient. The results indicated that patients with positive family history tend to have an aggravated course of ISSNHL. Further studies should help to confirm these results and to identify environmental or genetic factors leading to ISSNHL. This might support a better understanding of the aetiology of ISSNHL and offer new possibilities for prevention and therapy.

Association between Family History and Idiopathic Sudden Sensorineural Hearing Loss

“…When all data were statistically evaluated, a significant relationship was observed between ISSNHL and both the family medical history and the degree of kinship. The results of the present study are consistent with the data of Gäckler et al [31] in terms of the presence of ISSNHL and degree of kinship. A few previous studies have been published on this subject.…”

“…In the same study, the authors found that 10 patients had two relatives with a positive ISSNHL history [31] . In our study, the first-degree relatives of 29 (23.22%) patients and second-degree relatives of 12 (9.6%) patients had a history of ISSNHL.…”

“…Gäckler et al [31] reported a positive family medical history rate of 21.4% in their study of 186 patients diagnosed with ISSNHL.…”

Hereditary bilateral sudden sensorineural hearing loss

“…The possible role of genetic factors in SSNHL had been largely overlooked in the past, despite sporadic records of familial SSNHL cases (13). In general, genes and variants with autosomal recessive inheritance cause congenital or early onset (prelingual) deafness, whereas genes with autosomal dominant inheritance lead to progressive hearing loss often with delayed onset.…”

The Role of Soluble Adhesion Molecules and Cytokines in Sudden Sensorineural Hearing Loss

“…Recent SSNHL studies documented a positive family history for SSNHL, which might be a hint for genetic factors in this disease. 5 An increased blood viscosity 6 that might be related to elevated fibrinogen levels 7 in patients with SSNHL may support the vascular hypothesis. Other authors report that SSNHL cooccurring with hypertension, diabetes mellitus, or dyslipidemias seems to be associated with a higher prevalence of cerebral microangiopathy and with a slower hearing recovery.…”