title: Frequency of ear symptoms and hearing loss in ichthyosis: a pilot survey study.
abstract: Ichthyoses comprise a heterogeneous array of skin conditions resulting from impairment of cornification. Although ear structures can be affected, ear-related symptoms have never been investigated in patients with ichthyosis. In this pilot survey study, our aim was to determine the frequency of ear symptoms, hearing loss, and related medical interventions in patients with ichthyosis. Our secondary aim was to compare the frequency of these items according to age group. An online survey using Redcap was developed and posted online on the Foundation for Ichthyosis and Related Skin Types website for 6 months. Patients or parents of patients with ichthyosis were asked to complete the survey. Data analysis excluded patients with keratitis-ichthyosis-deafness syndrome and surveys that had fewer than two completed items. One hundred thirty-five unique surveys were used for data analysis. Of all participants, 80% reported ear pruritus, 66% reported trouble hearing, 29% reported frequent ear pain, 28% had abnormal hearing test results, and 16% had used hearing aids. Of the 88 participants who reported trouble hearing, 24 (27.3%) had never been to a hearing specialist. Significantly more participants older than 18 years of age (74%, 57/77) reported trouble hearing than participants age 18 years and younger (53%, 31/58; p = 0.02). The frequencies of other ear symptoms and hearing loss were not statistically significantly different between the age groups. Ear pruritus, ear pain, and hearing loss are important concerns in patients with all forms of ichthyosis in all age groups. Early diagnosis and intervention may improve the quality of life of patients with ichthyosis.
authors.affiliation: Dermatology Program, Boston Children's Hospital, Boston, Massachusetts; Department of Dermatology, Harvard Medical School, Boston, Massachusetts.
authors.forename: Jennifer T
authors.initials: JT
authors.lastname: Huang
authors.forename: Kaitlin
authors.initials: K
authors.lastname: Mallon
authors.forename: Shannon
authors.initials: S
authors.lastname: Hamill
authors.forename: Laurie A
authors.initials: LA
authors.lastname: Ohlms
authors.forename: Marilyn G
authors.initials: MG
authors.lastname: Liang
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D003638:Deafness / Q000453:epidemiology*; D004433:Earache / Q000453:epidemiology*; D005260:Female; D006306:Health Surveys; D034381:Hearing Loss / Q000453:epidemiology*; D006801:Humans; D016112:Ichthyosis Vulgaris / Q000453:epidemiology*; D017490:Ichthyosis, Lamellar / Q000453:epidemiology*; D007223:Infant; D008297:Male; D008875:Middle Aged; D010865:Pilot Projects; D011237:Predictive Value of Tests; D015995:Prevalence; D055815:Young Adult