Pendred syndrome

Treatment strategies in chronic ear disease

Editor:

I would like to share with your readers a few comments about two articles that appeared in the supplement to the June issue of EAR, NOSE, AND THROAT JOURNAL entitled, "Treatment strategies in chronic ear disease." [1,2] My comments might seem to be anecdotal rather than scientific, but they are based on almost 40 years of experience in our specialty. In my hands, the thinking expressed in this letter has worked well in the vast majority of cases.

Post-tympanostomy tube otorrhea. With regard to the topic covered by Myer, [1] I believe that those children who experience very early drainage from the tympanostomy tube are most likely experiencing some allergic reaction to the tube itself. Rarely are they infected, unless the parent has not undertaken safe ear protection measures postoperatively.

It makes little sense to me to use any topical antimicrobial drops. I find it hard to believe that any significant amount of these drops will reach the middle ear, especially if there is viscous fluid in the ear under pressure. In most cases, oral antibiotics are also a waste of time and money, and they can lead to bacterial resistance.

My initial approach is to once again review with the parents the accepted methods of keeping water out of the ear during bathing. I then try to gently suction out the ear through the tube and apply some topical antibiotic ointment in the canal--partly because it "feels good" and reduces the amount of canal irritation from the drainage. On occasion, I use a puff of antibiotic powder, mainly for its drying effect. If the child has evidence of allergic upper respiratory symptoms, I use a low dose of oral dexamethasone solution for 5 or 6 days.

If this regimen proves to be unsuccessful after four to six visits, I remove the tubes. I tell the parent that the child is allergic to the tubes and that they would do well to consider an allergy workup.

Chronic external otitis. In reference to the issues raised by Roland, [2] we all know that chronic external otitis manifests in many forms and that no single approach works for everyone. My basic thought is that this condition is actually a dermatitis that eventually becomes infected by a mixture of bacterial flora and fungi. There is certainly an allergic component, as well as an emotional component (anxiety). I believe our task is to restore the skin to normal and perhaps even to get a bit of cerumen to form again.

I try hard at each visit to meticulously clear the canal with small applicators and light suction. If I begin with peroxide, I use small dry applicators to clean out all debris and particles. Magnification is very helpful here. I then use a fine suction to remove any debris that I did not get with the applicators.

Once the ear canal is dry and clean, I apply a light coating of topical antibiotic and steroid ointment and follow that with a very light puff of triple-antibiotic powder (Chloromycetin [chloramphenicol], sulfanilamide, and amphotericin B).

The final step is to reassure the parents that the child will get well and the terrible itching will stop. I also point out that they are not "helping" me by using Q-Tips, match sticks, or bobby pins on their own and that they should forget about the wonder drops or garlic that Grandma used to use. I tell them to leave the ears alone and that I will give them another treatment in about a week. In some cases, I prescribe a 6-day course of oral prednisone to decrease the inflammatory response. It works.

Ellis C. Berkowitz, MD

Los Angeles

(1.) Myer CM III. Post-tympanostomy tube otorrhea. Ear Nose Throat J 2001;80(Suppl [Jun]):4-7.

(2.) Roland PS. Chronic external otitis. Ear Nose Throat J 2001;80(Suppl [Jun]:12-6.

Response

I greatly appreciate the trouble Dr. Berkowitz has taken to contribute to our understanding of post-tympanostomy tube otorrhea and chronic external otitis based on his 40 years of experience. As regards to his remarks on post-tympanostomy tube otorrhea, the idea that early-onset post-tympanostomy tube otorrhea is related to an allergic reaction to the substance of the tube is an unusual concept. Review of the literature indicates that cultures are generally positive and that appropriate antimicrobial therapy eliminates the otorrhea and that the child then retains the tube for many months drainage-free. This does not seem to me to be consistent with an allergic reaction which, I believe, would persist regardless of therapy.

Antimicrobial drops do penetrate through the lumen of the tube: There are now several studies demonstrating this, the most compelling of which are those that detect fluorosein in the nasopharynx after administration of fluorosein-containing topical ear drops. A number of those studies were done in children with middle ear effusion and draining tympanostomy tubes. There are now seven or more studies showing that surface swimming does not increase the incidence of post-tympanostomy tube drainage. Given the fact that swimming does not increase the incidence of post-tympanostomy tube otorrhea, it seems unlikely that bathing would be problematic.

As regards to chronic external otitis, I am in general agreement with Dr. Berkowitz. I only add that suction must be done extremely gently, as even the most minor trauma to the canal skin is likely to accelerate the sclerosing process.

Peter S. Roland, MD

Professor and Chairman

Otology/Neurotology/Skull Base Surgery

Pediatric Otology

The University of Texas

Southwestern Medical Center at Dallas

Do not overlook Pendred's syndrome in children with sensorineural hearing loss

Editor:

Pendred's syndrome is the result of an autosomal-recessive genetic defect in thyroxine synthesis. It is characterized by a sensorineural hearing loss (SNHL) and goiter, and it is responsible for 10% of all cases of hereditary deafness (incidence: 7.5 to 10 per 100,000 population.) [1-4] To draw attention to Pendred's syndrome in children with SNHL, we summarize our findings in five such children.

The five patients had been admitted to the pediatric endocrinology clinic at the Erciyes University Faculty of Medicine in Kayseri, Turkey, for an evaluation of goiter (table). The parents of patients 2 and 3 had noted their child's deafness during the newborn period, and the others had become aware of the deafness 6 months to 2 years before admission to our hospital. The presence of goiter had been first noticed 1 month to several years prior to admission. Patient 1 had been hospitalized for lower respiratory tract infection as a newborn, and he had undergone surgery for an inguinal hernia at the age of 4 years; three of his siblings had died during infancy. Patient 4 had 10 siblings, four of whom had died during infancy (we were unaware of the causes); three others had deafness.

Findings on thyroid imaging (ultrasonography or scintigraphy) and the results of thyroid hormone measurements are shown in the table. None of these patients had undergone a perchlorate discharge test because it had not been available. All patients were treated with levothyroxine. Patient 1 later underwent a subtotal thyroidectomy because of his large multinodular goiter; histopathologic analysis revealed that the goiter was dyshormonogenic.

In patients with Pendred's syndrome, congenital SNHL is present at birth; it is severe in half of all cases. Goiter becomes apparent during infancy or early childhood, but it is usually not accompanied by signs of thyroid insufficiency. The laboratory method for assessing iodide organification is the perchlorate discharge test, which produces a marked decline in the radioiodine content of the thyroid gland (to [less than or equal to] 10% of baseline value). Goiter is better treated medically than surgically. Exogenous hormone therapy can decrease the production of thyroid-stimulating hormone, which leads to a reduction in the size of the goiter. The hearing loss is not reversible. [1,2,5]

We wish to stress that Pendred's syndrome should be considered in all children who have SNHL. When appropriate, patients can be referred to a pediatric endocrinologist for further evaluation and treatment.

Huseyin Caksen, MD

Department of Pediatrics

Yuzuncu Yil University Faculty of Medicine

Van, Turkey

Selim Kurtoglu, MD

Saban Yuksel, MD

Ahmet Ciftci, MD

Mustafa Kendirci, MD

Department of Pediatrics

Erciyes University Faculty of Medicine

Kayseri, Turkey

(1.) Lafranchi S. Hypothyroidism. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. l6th ed. Philadelphia: W.B. Saunders, 2000:1698-704.

(2.) Fenichel GM. Clinical Pediatric Neurology: A Signs and Symptoms Approach. 3rd ed. Philadelphia: W.B. Saunders, 1997.

(3.) Kabakkaya Y, Bakan E, Yigitoglu MR, et al. Pendred's syndrome. Ann Otol Rhinol Laryngol 1993;102:285-8.

(4.) Sheffield VC, Kraiem Z, Beck JC, et al. Pendred syndrome maps to chromosome 7q21-34 and is caused by an intrinsic defect in thyroid iodine organification. Nat Genet 1996;12:424-6.

(5.) Aicardi J. Diseases of the Nervous System in Childhood. 2nd ed. London: Mac Keith Press, 1998.

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