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The Role of an Otolaryngologist in the Care of Ectodermal Dysplasia

The literature contains few references to the effects of the ectodermal dyspiasias (ED) on the normal functions of the ears, nose, and throat. Therefore the physician must rely upon clinical judgment and experience in treating similar pathologic conditions to formulate a treatment plan for patients with ED. Because the otolaryngologic manifestations of ED are secondary to maldevelopment of the exocrine glands, there is a decrease in the amount of the glands' secretions secondary to mucous gland hypoplasia. Because the underlying pathologic defect cannot be corrected, aiternative measures must be developed to minimize a patient's symptoms (1-3). Nasal obstruction is one of the most common complaints of individuals with ED. In addition to an overall lack of nasal secretions, the secretions that are produced are quite tenacious in consistency. As a result, mucous stasis and crusting are quite common, with frequent secondary infection of the nose and paranasai sinuses. Dryness commonly leads to vestibulitis and crusting in the anterior aspect of the nose, and this frequently predisposes patients with ED to epistaxis. As in the general population, this occurs most often from the anterior nasal septum. The most effective method of dealing with the altered nasal physiology in patients with ED is to provide additional humidification. The home environment can be modified with home or room humidifiers, or both. Locally, normal saline nose drops or commercial solutions such as Ocean, Ayr, and Alkaloi are beneficial and can be used as frequently as necessary. One must caution patients against using decongestant sprays on a regular basis since they are intended for temporary symptomatic relief of upper airway congestion and not to be used as a source of humidification. If nasal crusting and concretions develop in spite of adequate humidification efforts, crusts may be removed mechanically either by the patient or by the physician. After removing the crusts, application of a biand antimicrobial ointment on a cotton-tipped swab to the anterior nasal septum is an effective way of improving nasal lubrication. Similar to the general population, patients with ED can have anatomic deformities that cause nasal obstruction. The term "saddle nose" identifies a type of deformity in which the dorsum of the nose is depressed. Although it is often associated with ED, a saddle nose is not specific for these patients and can be seen in other congenital conditions. In addition, it can result from trauma or infection of the nasal septum, or as one of the sequelae of nasal surgery. Surgical correction of a saddle nose is quite intricate and requires the implantation of a bone graft to restore the nasal profile. Surgical correction of a deviated septum (septoplasty) generally facilitates nasal breathing. Patients with ED, however, may not obtain optimal results from such a procedure. Although septoplasty may