More than two-thirds of patients with ichthyosis, a hereditary skin disease characterized by dry, thick, scaly or flaky skin, reported hearing problems, according to data from a survey of 135 patients ranging in age from 5 months to 80 years.
Dr. Jennifer T. Huang of Boston’s Children’s Hospital reported the study’s results at the annual meeting of the Society for Pediatric Dermatology. About 80 percent of the survey’s subjects reported ear pruritus, 66 percent had trouble hearing, 29 percent frequent ear pain, 28 percent abnormal hearing test results and 16 percent had used hearing aids. Of the 88 patients who reported trouble hearing, 27 percent had never seen a hearing specialist.
“Early diagnosis and intervention, by both pediatric dermatologists and pediatricians may improve quality of life,” Huang reported at the society’s annual meeting. “Regular ear canal debridement and hearing tests should become routine care for patients reporting ear symptoms.”
What Exactly is Ichthyosis?
Ichthyosis includes any number of heterogeneous skin disorders characterized by dry, scaling skin. According to Huang, otologic issues can be attributed to abnormal keratinization of the external ear canals and tympanic membranes or accumulation of emollients within the ear canals.
Sufficient build-up plugs the ear canals, resulting in conductive but temporary hearing loss. Ear structures can be affected but ear-related symptoms have never been investigated in ichthyosis patients.
The Foundation for Ichthyosis & Related Skin Types (FIRST) posted the pilot survey on their website for six months. Dr. Huang and her colleagues analyzed the data for 135 of 148 respondents. The other 13 patients either had keratisis ichthyosis deafness (KID) syndrome or did not complete the survey beyond one item.
What is the Most Common Form of Ichthosis?
The most common type of ichthosis experienced by 30 percent of the survey’s patients, lamellar ichthyosis is characterized by extensive scaling of the skin, generally concentrated around joints in areas like the groin, the armpits, the inside of the elbow and the neck. While 17 percent of the ichthyosis cases were unknown, 13 percent experienced vulgaris ichthyosis, 12 percent X-linked ichthyosis, 10 percent congenital ichyosiform erythroderma and 10 percent epidermolytic hyperkeratosis.
The patients’ average age was 27 years, with 43 percent younger than 18 years. According to Huang, adult patients, or those 18 years or older, were more likely than pediatric patients to report trouble hearing, 74 percent compared to 53 percent.
However, there were no significant difference between the older and younger age groups in the reported frequency of rubbing and scratching, ear pain, abnormal hearing test results or use of hearing aids.
Of the 75 patients who had a previous hearing test, about half, or 51 percent were found to have hearing deficits. Half of the patients with abnormal results also reported using hearing aids. Two-thirds of the patients reported previous ear cleaning.
There were no significant differences in frequency of trouble hearing, ear pruritus and ear pain among the ichthyosis types.
“Patients with all forms of ichthyosis, across all age groups, are affected,” said Dr. Huang, who called for further studies using objective tools to measure hearing loss and intervention assessments.
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