Syphilis of the ear is for obvious reasons not often discoverable until the patient has reached an age at which interference with the function of hearing becomes a noticeable phenomena. The only symptom likely to attract attention during the stage of inherited syphilis which we are now considering is a catarrh of the middle ear, which may have for its starting-point some inflammation, ulceration, or mucous patch of the pharynx, causing a temporary or permanent occlusion of the orifices of the Eustachian tubes.86 This may lead to perforation of the membrana tympani, purulent infiltration of the mastoid cells, etc., and when accompanied by an otorrhoea which attracts attention to the ear will be easily discovered by the physician. These cases are, however, exceptional, otorrhoea only being present in nine out of Hutchinson and Jackson's87 one hundred cases of inherited syphilis, and consequently but little is known about the frequency or gravity of lesions of the auditory apparatus in the secondary stage of this form of syphilis.88 The changes which occur later on are chiefly those which involve either the nerves themselves or their distribution in the labyrinth.
86 Bäumler, Ziemssen's Cyclopædia, vol. iii. p. 226.
87 Hutchinson and Hughlings Jackson, Med. Times and Gaz., Nov. 23, 1861.
88 Schwartze (quoted by Hill and Cooper) found also that otorrhoea was a rare complication in deafness from syphilis.
The affections of the middle ear and Eustachian tube are said to be contemporaneous with the keratitis which appears in the neighborhood of puberty,89 while those of the nerve are somewhat later in point of time, and are almost always conjoined with retinitis, choroiditis, and optic neuritis. As usual when investigating or describing any subject relating to syphilis, Mr. Hutchinson's opinion and observation must be detailed. In 1863 he wrote90 that it was only recently that he had thought of specially investigating the disorders of hearing in reference to hereditary taint, having had his attention called to a peculiar form of deafness, usually symmetrical, passing rapidly through its different stages and unaccompanied by any marked degree of pain or any external disease. He then reported eighteen cases of which he had notes. The oldest of these patients was twenty-seven, the youngest eight—the average time of development of the deafness from twelve to fifteen. Although the membrana tympani was in no instance quite normal, in none were there found adequate changes to account for the deafness. In all the Eustachian tubes were pervious. In nearly all the disease was symmetrical. This fact, together with the absence of discoverable lesions of the external or middle ear, seems to point conclusively to disease of the nerves themselves, or at least to a central cause.91 He adds: "With regard to the prognosis of heredito-syphilitic deafness, I believe that it is very unfavorable. When the disease was progressive I have rarely witnessed any permanent improvement or arrest. In most it has gone on to total loss of hearing, and this in several instances in spite of the cautious use of specific remedies almost from the beginning. From six months to a year would appear to be the usual time required for the completion of the process and the entire abolition of the function."92
89 Purves, Guy's Hospital Reports, 1875, p. 564; Pritchard, British Medical Journal, April 21, 1877.
90 Clinical Memoirs on Certain Diseases of the Eye and Ear consequent on Inherited Syphilis, London, 1863, pp. 182, 183.
91 In the Lancet for Jan. 16, 1875, he reports a case of total deafness in a young woman of seventeen which had come on in ten months without pain or otorrhoea. He believes the disease of the organ of hearing to be parallel with those cases of choroiditis disseminata or of optic neuritis in which blindness is produced without pain or any external evidence of inflammation, and which are distinctly and positively associated with inherited syphilis.
92 Mr. Hinton, in his edition of Toynbee's work on Diseases of the Ear, states that at Guy's Hospital, of his aural patients, one in twenty is affected with deafness due to heredito-syphilis; that it usually makes its appearance between the tenth and sixteenth years; and that the great majority of the cases which he has seen have been females. He adds: "Patients suffering from this disease may, as a rule, at least when young, be at once distinguished by the amount of deafness which they exhibit. I know no other affection except fever which in a person under twenty brings on a deafness so rapidly and so nearly complete. In the course of a few weeks a girl previously hearing well will, without pain or known cause, become unable to distinguish words." In one of Dalby's cases total deafness came on in three weeks, previous to which hearing was normal. According to Pierce, the deafness is most apt to manifest itself between eleven and eighteen years of age. Troeltsch says that "l'audition du diapason par le vertex" is lost at an early date after the beginning of the disease, and that there are also often concomitant affections of the nose and pharynx.
Dalby93 is said to regard syphilis as, next to scarlatina, the most fruitful cause of deaf-mutism as it occurs in children born with good hearing powers. "The patient usually becomes deaf in early childhood—after he begins to talk—or between this period and puberty."94