Pediatrics: Hearing Help

The telltale rash of German measles (rubella) can come and go unseen during a night's sleep. In fact, the disease is generally so mild that a nationwide epidemic of it three years ago caused no panic. An estimated 30,000 pregnant women were among those infected, however, and rubella can wreak tragic damage in unborn children. For one of every two rubella babies, that damage includes at least a partial loss of hearing. "The deafness we are seeing now—the aftermath of the epidemic—is more severe than anyone anticipated," says Dr. Fred Linthicum Jr. of the children's division of the Los Angeles Otological Medical Group. "We are encountering greater and more severe losses than doctors have ever seen before."

No one is sure exactly how the rubella does its disabling work, but one result is the stunted growth of thousands of microscopic hair cells on the acoustic nerve in the recesses of the inner ear (see diagram). Doctors recently proved, by passing a wire under the hair cells and stimulating the nerve, that there is no nerve damage. But Dr. Edgar Lowell of the John Tracy Clinic* points out that "we still haven't cracked the neural code that transmits messages from the hair cells to the hearing nerve below." The ear conceals other mysteries as well, and there is no surgical or other cure for rubella deafness at the moment. As far as doctors can tell, the child's hearing loss will get no worse—or better—throughout his life.

Early Aid. But if cure is not possible, adjustment to partial hearing is—and "early diagnosis is crucial," says Johns Hopkins' Dr. John Bordley. Sadly, rubella makes that difficult. The disease can also cause mental retardation and slight brain damage. In a child's first year or two, the symptoms of both ailments are similar to those of deafness: the child fails to associate sounds with their sources and respond directly to external stimuli. He will also not learn to talk on schedule. But simple tests by doctors can usually discover whether the cause of such symptoms is deafness, and there is now a new tool for more difficult diagnoses, a computerized electroencephalogram. Electrodes are taped to the infant's head to measure brainwave responses to sounds. The responses are averaged by the computer, and the results are compared with those of a normal child.

Once the case is diagnosed, the treatment is liable to be distressingly traditional and only mildly effective. As Dr. Harold F. Schuknecht of the Massachusetts Eye and Ear Infirmary puts it: "You hang a hearing aid on 'em, give them lip reading and special training."

Often, special training means being packed off to a school for the deaf where the child is forced into awareness that he is "different" and less than normal. Also, the hearing aid is frequently not put on until the age of two, when the child has already begun to accommodate himself to a special closed world.