hereditary ear fistula
Introduction
Introduction to hereditary deafness Hereditary auricular hair (hereditaryauricular finistula), also known as earpit (earpit), refers to the ear asymptomatic abnormal small hole or small fistula. This is a common autosomal dominant genetic disease caused by poor fusion of the six mound-like nodules of the first and second zygomatic arches during the embryonic period or the first sulcus occlusion. The incidence rate is 1.2%, which can occur on one or both sides. The ratio of one side to both sides is about 4:1. The ratio of men to women is about 17:1. basic knowledge The proportion of the disease: the probability of the population is 0.03% Susceptible people: no special people Mode of infection: non-infectious Complications: otitis media
Cause
Hereditary deafness
Cause:
Part of the autosomal dominant inheritance, deafness or anterior deafness is caused by the failure of the first and second zygomatic arches to be well combined, most commonly in the upper area of the ear wheel or in the front, more common in men, the same family Different members can have bilateral or unilateral deafness.
Pathogenesis
The pathogenesis is still unclear.
Prevention
Hereditary deafness prevention
The disease belongs to chromosomal diseases, and the cause of chromosomal abnormalities is unclear. It may have certain correlation with environmental factors, genetic factors, dietary factors, and mood and nutrition during pregnancy, so this disease cannot be directly prevented. Regular examination should be done during pregnancy. If the child has a tendency to develop abnormalities, chromosome screening should be done in time, and abortion should be performed in time to avoid the birth of the diseased child. In daily life, we should pay attention to nutrition and rationality. Try to diversify foods as much as possible. Eat high-protein, multi-vitamins, low-fat animal fats, digestible foods, fresh fruits and vegetables, and do not eat old-fashioned or irritating things. Grilled, marinated, fried, salty foods, staple foods and coarse grains to ensure nutritional balance.
Complication
Hereditary deafness complications Complications otitis media
Occasionally accompanied by recurrent otitis media. Otitis media is an inflammatory lesion involving all or part of the structure of the middle ear (including the eustachian tube, tympanic cavity, sinus sinus and mastoid air chamber), which occurs in children. Can be divided into two categories of non-suppurative and suppurative. Non-suppuratives include secretory otitis media, air-damaged otitis media, etc. Suppurative patients have acute and chronic points. Specific inflammation is too rare, such as tuberculous otitis media.
Symptom
Hereditary deafness symptoms Common symptoms Pre-auricular lymphadenopathy Granuloma nodules secondary infection
A small hole in the upper part of the ear wheel or in front of it, asymptomatic, sometimes intermittently expelling a white cheese-like liquid in infancy or childhood, including cell debris, if there is a secondary infection, it can cause lymph node suppuration and Swelling can also form small granulomatous nodules in the fistula. The cervical deafness from the first arch is very rare. It can extend from the external auditory canal to the neck below the mandibular angle. Continued to middle age, occasionally accompanied by recurrent otitis media.
Examine
Hereditary deafness examination
This is to be combined with the actual situation of the patient to determine the inspection matter. Pathological view of the naked eye, the surface of the skin specimen has a fistula mouth, and the fistula is a branched or curved blind tubular structure. The wall of the tube is fibrous connective tissue, covered with stratified squamous epithelium, or pseudo-stratified fibrous columnar epithelium, sometimes both, or accompanied by chronic inflammation. There are elastic cartilage in the deep wall. The outer mouth is a small concave on the skin, and the squeeze may have a small amount of white sebum-like substance, which is slightly odorous.
Diagnosis
Diagnosis of hereditary deafness
diagnosis
According to family history and clinical manifestations, it can be diagnosed.
Differential diagnosis
Generally not confused with other diseases.
