MILWAUKEE – More than two-thirds of patients with ichthyosis reported hearing problems, based on data from a survey of 135 patients ranging in age from 5 months to 80 years.
Overall, 80% of the survey respondents had ear pruritus, 66% had trouble hearing, 29% reported frequent ear pain, 28% had abnormal hearing test results, and 16% had used hearing aids.
Notably, of the 88 patients who reported trouble hearing, 27% had never seen a hearing specialist, Dr. Jennifer T. Huang reported at the annual meeting of the Society for Pediatric Dermatology.
"Early diagnosis and intervention, by both pediatric dermatologists and pediatricians, may improve quality of life," she said.
She also recommended that "regular ear canal debridement and hearing tests should become routine care for patients reporting ear symptoms."
Ichthyosis includes a heterogeneous array of skin disorders characterized by dry, scaling skin, and otologic issues may be attributed to abnormal keratinization of the external ear canals and tympanic membranes and/or accumulation of emollients within the ear canals, said Dr. Huang of Boston Children’s Hospital, Mass. Sufficient build-up causes the ear canals to become plugged, resulting in conductive (though not permanent) hearing loss. While ear structures can be affected, ear-related symptoms have never been investigated in patients with ichthyosis, according to the authors of the award-winning research poster.
The pilot survey was posted on the Foundation for Ichthyosis & Related Skin Types (FIRST) website for 6 months, and Dr. Huang and her colleagues analyzed the data for 135 of 148 respondents. Patients with keratitis ichthyosis deafness (KID) syndrome and surveys with fewer than two completed items were excluded from the analysis.
Lamellar ichthyosis was the most common type of ichthyosis (30%), followed by unknown (17%), vulgaris (13%), X-linked (12%), congenital ichthyosiform erythroderma (10%), and epidermolytic hyperkeratosis (10%). The patients ranged in age from 5 months to 80 years, with an average age of 27 years; 43% were younger than 18 years.
Adult patients, defined as those 18 years and older, were significantly more likely than pediatric patients to report trouble hearing (74% vs. 53%), Dr. Huang reported.
There were no significant differences, however, between older and younger patients in the reported frequency of rubbing/scratching (79% vs. 74%), ear pain (22% vs. 38%), abnormal hearing test results (30% vs. 26), and use of hearing aids (17% vs. 14%).
However, parents who completed the survey for their infant or child may not have been able to fully report the child’s hearing difficulties, she noted.
Of the 75 patients who had a previous hearing test, half (51%) were found to have hearing deficits. Half of those patients who had abnormal results also reported using hearing aids. Two-thirds of patients reported previous ear cleaning (66%).
Finally, the frequency of trouble hearing, ear pruritus, and ear pain was not significantly different among types of ichthyosis.
"Patients with all forms of ichthyosis, across all age groups, are affected," noted Dr. Huang, who called for further studies using objective tools to measure hearing loss and intervention assessments.
Dr. Huang reported having no financial disclosures, but acknowledged the assistance of the Foundation for Ichthyosis & Related Skin Types in the execution of the survey.