Prevalence of hearing loss and clinical otologic manifestations in patients with 22q11.2 deletion syndrome: a literature review

Abstract

Background

Hearing loss and otitis media are frequently reported in patients with 22q11.2 deletion syndrome.

Objective of review

Our objective was to review the current literature on the prevalence of hearing loss and otologic manifestations in patients with 22q11.2 deletion syndrome.

Type of review

Systematic review.

Search strategy

We conducted a systematic search in PubMed and Embase combining the term '22q11.2 deletion syndrome' and synonyms with 'hearing loss' and 'otologic manifestations' and synonyms.

Evaluation method

We screened title/abstract and full text of all retrieved articles on predefined in- and exclusion criteria. The remaining articles were assessed on risk of bias. Outcome measures included the prevalence of hearing loss and otologic manifestations such as otitis media.

Results

Our search yielded 558 unique studies of which a total of 25 articles were included for critical appraisal and data extraction. 21 studies reported on hearing loss and 21 studies on otologic manifestations. The prevalence of hearing loss varied from 6.0% to 60.3%, where in most studies conductive hearing loss was most prevalent. Rates of recurrent or chronic otitis media varied from 2.2% to 89.8%.

Conclusion

Although a very broad range in prevalences is reported in different studies, hearing loss and recurrent or chronic otitis media are frequently present. Regular check-ups and audiometric testing is advised in patients with 22q11.2 deletion syndrome.

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