Date: 03/24/2014

Hearing Loss, Ear Pain in Ichthyosis Patients

Huang JT, Mallon K, Hamill S, Ohlms LA, Liang MG.
Pediatr Dermatol. 2014 Mar 6. doi: 10.1111/pde.12292. [Epub ahead of print]

During 2012-2013, the doctors at Boston Children’s Hospital, Harvard Medical School conducted a pilot survey study using FIRST’s website to obtain information about ichthyosis and its ear-related symptoms.  The survey was posted for six months, attracting 135 participants.

“While ear problems are well-recognized in patients with ichthyosis, ear-related symptoms have never been systematically investigated in those patients,” said Dr. Jennifer Huang, the primary investigator in the research project.  “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 by age group.”

During the summer of 2013, at the annual Society for Pediatric Dermatology meeting in Milwaukee, a complete analysis of the data was released.  Among other factors, the data revealed that of all participants, 80% reported ear pruritus (itch), 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 > 18 years of age (74%, 57/77) reported more trouble hearing than participants ≤ 18 years of age (53%, 31/58).

Moreover, these recently released results did, in fact, support the theory that ear pruritus (itching), ear pain, and hearing loss are significant issues for patients with all forms of ichthyosis, across all age groups. 

As a precautionary measure to prevent hearing and ear pain issues, Dr. Huang recommends that both patient and doctor take a proactive role in thwarting more serious issues.  “All patients with generalized ichthyosis should be asked by their pediatrician or their primary doctor if they have hearing problems.  If hearing problems exist, a careful evaluation of their external ear canal and a hearing exam should be performed,” Huang said.  

She also encourages FIRST members affected by ichthyosis, who are not having routine ear exams, to consider requesting an exam. “Ask your doctor the proper procedure for manual cleaning. If needed ,ears can be routinely cleaned at the doctor’s office, as well as at home. Also ear wax softeners can be helpful.”

Dr. Huang and her team plan to expand their research into phase 2. “Next step is meeting with patients, in-person, to conduct hearing tests and interview them one-on-one. This will further validate our data.” 

Find out the best ways to care for your ears.

A full article regarding this research can be found at:

*Cornification (synonym keratinization): n. conversion into keratin. Ref.

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