The pattern is open!

The New England Journal reported such a clinical case of neurosyphilis diagnosed in the Department of Rheumatology and Immunology on February 10, 2022

A 55-year-old man presented to the Rheumatology and Immunology Department with bilateral hearing loss and red eyes

8 weeks ago

Pain in the left temple and a similar headache during a shingles attack 15 years ago

4 weeks ago

Hearing loss in left ear during flight

Physical examination: both eyes were red, the left and right auricles and external auditory canal were normal, the right tympanic membrane was also normal, the left tympanic membrane was retracted, and there was no middle ear effusion

The patient was prescribed a12-day tapering course of oral prednisone .

 

After 4 weeks of treatment

Otolaryngology follow-up results: Hearing loss had progressed, and redness of the eyes initially decreased after starting prednisone but recurred after prednisone was discontinued

Physical examination: red eyes, no spontaneous nystagmus or gait ataxia

3 days later

Three days later, the patient was seen in the rheumatology outpatient clinic

Chief Complaint : Hearing loss in both ears and redness in both eyes

Has a history of irritable bowel syndrome , diverticulosis, gastritis, allergic rhinitis, and asthma

On examination, the patient was doing well

The rest of the examination, including evaluation of the skin, joints, and nervous system, was normal

 On the ophthalmic evaluation, the patient reported no blurred vision, floaters, eye pain, pruritus, or photophobia

Schematic diagram of symptoms described by patients

 

The main symptoms of this 55-year-old male patient were progressive hearing loss, imbalance, and ocular inflammation

On the basis of this series of findings, differential diagnosis will focus on disorders that have the potential to cause temporal pain, asymmetric sensorineural hearing loss, and ocular inflammation

 

In conclusion, there are many possibilities for systemic disease associated with eye and ear involvement and systemic signs and symptoms
.
The patient's diagnosis of Cogan syndrome or syphilis was ultimately considered
.
Considering the feasibility of the diagnosis of syphilis, a lumbar puncture with cerebrospinal fluid (CSF) analysis was chosen to look for pleocytosis, and the Venereal Disease Research Laboratory (VDRL) test and the Fluorescent Treponemal Antibody Absorption (FTA-ABS) test were performed
.

Test result :

Luminescent microparticle immunoassays: IgM(+) and IgG(+); the patient had a positive rapid plasma reagin (RPR) titer of 1:512, as well as a positive non-Treponema pallidum test
.

Analysis of the patient's cerebrospinal fluid showed a colorless, non-turbid fluid with 21 nucleated cells per cubic millimeter, 54% neutrophils, 22% lymphocytes, and 24% monocytes, with a total protein level of 83 g/dL
.
The CSF VDRL test was reactive at 1:2; this result supports the diagnosis of neurosyphilis
.

The answer is already obvious, the patient's diagnosis: neurosyphilis
.

So why does neurosyphilis cause hearing loss in patients? What are the possible pathophysiological processes?

There is evidence of hematogenous spread of Treponema pallidum throughout the body and possibly into the temporal bone
.
In the later stages of syphilis, CSF enters the inner ear as it passes through the inner auditory canal or the cochlear aqueduct
.
The inner ear is immunoreactive and Treponema pallidum should elicit humoral antibody responses and activate innate immunity through proinflammatory cytokines and CD4+ and CD8+ T cells
.
All the necessary components of this response are located in the inner ear in the area of ​​the endolymphatic sac
.

Does continued glucocorticoid use after syphilis diagnosis and treatment increase the likelihood of hearing recovery?

Dr.
Harris: Patients with symptoms of various forms of rapidly progressive sensorineural hearing loss are often treated early with high-dose corticosteroids, sometimes intratympanic corticosteroids, which may reverse hearing loss and dizziness
.
Treatment of neurosyphilis with antibiotics alone may lead to infection control, but glucocorticoids are required to reduce inflammation in the inner ear and prevent irreversible fibrosis and osteogenesis
.

Therefore, subsequent glucocorticoid therapy will be considered if the patient has received long-term antibiotic therapy consistent with the recommendations of the Centers for Disease Control and Prevention
.

The New England Journal reported such a clinical case of neurosyphilis diagnosed in the Department of Rheumatology and Immunology on February 10, 2022
.

The New England Journal reported such a clinical case of neurosyphilis diagnosed in the Department of Rheumatology and Immunology on February 10, 2022
.
New England Journal of Immunization

A 55-year-old man presented to the Rheumatology and Immunology Department with bilateral hearing loss and red eyes
.
love review

A 55-year-old man presented to the Rheumatology and Immunology Department with bilateral hearing loss and red eyes
.
love review

8 weeks ago

8 weeks ago8 weeks ago

Pain in the left temple and a similar headache during a shingles attack 15 years ago
.
At the time, doctors speculated that the temple pain was caused by herpes zoster and prescribed a one-week course of valacyclovir
.
There were no skin symptoms and headaches were relieved during this process
.

Pain in the left temple and a similar headache during a shingles attack 15 years ago
.
At the time, doctors speculated that the temple pain was caused by herpes zoster and prescribed a one-week course of valacyclovir
.
There were no skin symptoms and headaches were relieved during this process
.

4 weeks ago

4 weeks ago4 weeks ago

Hearing loss in left ear during flight
.
The hearing loss persisted , and the patient began to experience intermittent tinnitus in the left ear and red eyes
.
He went to the otolaryngologist
.
The patient had no dizziness, otorrhea, rhinitis, or sore throat
.

Hearing loss in left ear during flight
.
The hearing loss persisted , and the patient began to experience intermittent tinnitus in the left ear and red eyes
.
He went to the otolaryngologist
.
The patient had no dizziness, otorrhea, rhinitis, or sore throat
.

Physical examination: both eyes were red, the left and right auricles and external auditory canal were normal, the right tympanic membrane was also normal, the left tympanic membrane was retracted, and there was no middle ear effusion
.
The results of the Weber test were inconsistent
.
Bilateral Rinne test was positive, and air conduction was greater than bone conduction
.
There was no sinus tenderness
.
Nasopharyngoscopy revealed a left-sided septum; the posterior portion of the nasopharynx was normal
.
Other examinations, including cranial nerve examination, were normal
.
The audiogram showed mild-to-severe sensorineural hearing loss with tilted ears in both ears (Fig.
1A)
.
The results were asymmetric at low frequencies, with more severe hearing loss in the left ear than in the right ear in the 125 to 2000 Hz range
.
The word recognition rate was 96% for the right ear and 84% for the left ear (reference range, 80 to 100)
.
Tympanometry in both ears was normal
.

Physical examination: Physical examination: both eyes were red, the left and right auricles and external auditory canal were normal, the right tympanic membrane was also normal, the left tympanic membrane was retracted, and there was no middle ear effusion
.
The results of the Weber test were inconsistent
.
Bilateral Rinne test was positive, and air conduction was greater than bone conduction
.
There was no sinus tenderness
.
Nasopharyngoscopy revealed a left-sided septum; the posterior portion of the nasopharynx was normal
.
Other examinations, including cranial nerve examination, were normal
.
The audiogram showed mild-to-severe sensorineural hearing loss with tilted ears in both ears (Fig.
1A)
.
The results were asymmetric at low frequencies, with more severe hearing loss in the left ear than in the right ear in the 125 to 2000 Hz range
.
The word recognition rate was 96% for the right ear and 84% for the left ear (reference range, 80 to 100)
.
Tympanometry in both ears was normal
.

The patient was prescribed a  12-day tapering course of oral prednisone .

The patient was prescribed a  12-day tapering course of oral prednisone .

 

 

After 4 weeks of treatment

After 4 weeks of treatment After 4 weeks of treatment

Otolaryngology follow-up results: Hearing loss had progressed, and redness of the eyes initially decreased after starting prednisone but recurred after prednisone was discontinued
.

Otolaryngology follow-up results: Hearing loss had progressed, and redness of the eyes initially decreased after starting prednisone but recurred after prednisone was discontinued
.

Physical examination: red eyes, no spontaneous nystagmus or gait ataxia
.
Sinusoidal gaze tracking works fine
.
The left head thrust test was positive, suggesting left vestibular hypofunction
.
Romberg test results were normal
.
The Futian step test was positive, and the patient turned to the left, suggesting left vestibular hypofunction
.
The tympanic measurements were normal (Fig.
1B)
.
The patient was referred for urgent rheumatic evaluation
.

Physical examination: Physical examination: red eyes, no spontaneous nystagmus or gait ataxia
.
Sinusoidal gaze tracking works fine
.
The left head thrust test was positive, suggesting left vestibular hypofunction
.
Romberg test results were normal
.
The Futian step test was positive, and the patient turned to the left, suggesting left vestibular hypofunction
.
The tympanic measurements were normal (Fig.
1B)
.
The patient was referred for urgent rheumatic evaluation
.

3 days later

after 3 days after 3 days

Three days later, the patient was seen in the rheumatology outpatient clinic
.

Three days later, the patient was seen in the rheumatology outpatient clinic
.

Chief Complaint : Hearing loss in both ears and redness in both eyes
.
Fatigue and intermittent body pain for almost 2 months and lost 5kg in 6 months
.
No history of oral or genital lesions; 6 weeks ago, he developed a nonpruritic, patchy rash on his trunk following influenza vaccination that resolved rapidly with oral antihistamines
.

Chief Complaint : Hearing loss in both ears and redness in both eyes
.
Fatigue and intermittent body pain for almost 2 months and lost 5kg in 6 months
.
No history of oral or genital lesions; 6 weeks ago, he developed a nonpruritic, patchy rash on his trunk following influenza vaccination that resolved rapidly with oral antihistamines
.
chief complaint

Has a history of irritable bowel syndrome , diverticulosis, gastritis, allergic rhinitis, and asthma
.
Medications include intranasal and inhaled fluticasone, vitamin B12, and omeprazole
.
No known drug allergies
.
The patient and his wife lived in an urban area in New England
.
No travel history
.
The patient is a retired construction worker
.
He did not smoke, drank very little and did not use illicit drugs
.
 

Has a history of irritable bowel syndrome , diverticulosis, gastritis, allergic rhinitis, and asthma
.
Medications include intranasal and inhaled fluticasone, vitamin B12, and omeprazole
.
No known drug allergies
.
The patient and his wife lived in an urban area in New England
.
No travel history
.
The patient is a retired construction worker
.
He did not smoke, drank very little and did not use illicit drugs
.
irritable bowel syndrome 

On examination, the patient was doing well
.
Body temperature 36.
9°C, blood pressure 115/79 mm Hg, heart rate 95 beats/min, respiratory rate 15 beats/min, oxygen saturation 100% while breathing ambient air
.
Red eyes
.
Finger rub test showed severe hearing loss in both ears
.
A positive Romberg test suggests vestibular or cerebellar dysfunction
.

On examination, the patient was doing well
.
Body temperature 36.
9°C, blood pressure 115/79 mm Hg, heart rate 95 beats/min, respiratory rate 15 beats/min, oxygen saturation 100% while breathing ambient air
.
Red eyes
.
Finger rub test showed severe hearing loss in both ears
.
A positive Romberg test suggests vestibular or cerebellar dysfunction
.

The rest of the examination, including evaluation of the skin, joints, and nervous system, was normal
.
Blood levels of electrolytes and glucose were normal, as were liver and kidney function tests
.
Differential complete blood counts were normal
.
The erythrocyte sedimentation rate was 55 mm per hour (reference range, 0 to 13), and the C-reactive protein level was 12.
3 mg/L (reference value, <8.
0)
.

The rest of the examination, including evaluation of the skin, joints, and nervous system, was normal
.
Blood levels of electrolytes and glucose were normal, as were liver and kidney function tests
.
Differential complete blood counts were normal
.
The erythrocyte sedimentation rate was 55 mm per hour (reference range, 0 to 13), and the C-reactive protein level was 12.
3 mg/L (reference value, <8.
0)
.

 On the ophthalmic evaluation, the patient reported no blurred vision, floaters, eye pain, pruritus, or photophobia
.
The visual acuity was normal in both eyes
.
The right cornea had a small amount of needle-like epithelial deposition, surrounded by a punctate epithelial erosion ring, and there were several small punctate epithelial erosion confluence areas in the center, without infiltration
.
There is a circle of punctate epithelial erosions around the left cornea, and there are several confluent areas of punctate epithelial erosions in the center without infiltration
.
There was evidence of anterior uveitis, 7 cells per high-power field in the right anterior chamber and 6 to 7 cells per high-power field in the left anterior chamber
.

 On the ophthalmic evaluation, the patient reported no blurred vision, floaters, eye pain, pruritus, or photophobia
.
The visual acuity was normal in both eyes
.
The right cornea had a small amount of needle-like epithelial deposition, surrounded by a punctate epithelial erosion ring, and there were several small punctate epithelial erosion confluence areas in the center, without infiltration
.
There is a circle of punctate epithelial erosions around the left cornea, and there are several confluent areas of punctate epithelial erosions in the center without infiltration
.
There was evidence of anterior uveitis, 7 cells per high-power field in the right anterior chamber and 6 to 7 cells per high-power field in the left anterior chamber
.

Schematic diagram of symptoms described by patients

 

The main symptoms of this 55-year-old male patient were progressive hearing loss, imbalance, and ocular inflammation
.
The patient had a 6-month history of weight loss, a 2-month history of body pain and fatigue, and a history of a non-pruritic rash on the trunk, and an 8-week history of headache involving the left temple
.
Erythrocyte sedimentation rate and C-reactive protein levels were elevated during the assessment period
.

The main symptoms of this 55-year-old male patient were progressive hearing loss, imbalance, and ocular inflammation
.
The patient had a 6-month history of weight loss, a 2-month history of body pain and fatigue, and a history of a non-pruritic rash on the trunk, and an 8-week history of headache involving the left temple
.
Erythrocyte sedimentation rate and C-reactive protein levels were elevated during the assessment period
.

On the basis of this series of findings, differential diagnosis will focus on disorders that have the potential to cause temporal pain, asymmetric sensorineural hearing loss, and ocular inflammation
.

On the basis of this series of findings, differential diagnosis will focus on disorders that have the potential to cause temporal pain, asymmetric sensorineural hearing loss, and ocular inflammation
.
diagnosis

 

In conclusion, there are many possibilities for systemic disease associated with eye and ear involvement and systemic signs and symptoms
.
The patient's diagnosis of Cogan syndrome or syphilis was ultimately considered
.
Considering the feasibility of the diagnosis of syphilis, a lumbar puncture with cerebrospinal fluid (CSF) analysis was chosen to look for pleocytosis, and the Venereal Disease Research Laboratory (VDRL) test and the Fluorescent Treponemal Antibody Absorption (FTA-ABS) test were performed
.

In conclusion, there are many possibilities for systemic disease associated with eye and ear involvement and systemic signs and symptoms
.
The patient's diagnosis of Cogan syndrome or syphilis was ultimately considered
.
Considering the feasibility of the diagnosis of syphilis, a lumbar puncture with cerebrospinal fluid (CSF) analysis was chosen to look for pleocytosis, and the Venereal Disease Research Laboratory (VDRL) test and the Fluorescent Treponemal Antibody Absorption (FTA-ABS) test were performed
.

Test result :

Test Results Test Results :

Luminescent microparticle immunoassays: IgM(+) and IgG(+); the patient had a positive rapid plasma reagin (RPR) titer of 1:512, as well as a positive non-Treponema pallidum test
.

Luminescent microparticle immunoassays: IgM(+) and IgG(+); the patient had a positive rapid plasma reagin (RPR) titer of 1:512, as well as a positive non-Treponema pallidum test
.

Analysis of the patient's cerebrospinal fluid showed a colorless, non-turbid fluid with 21 nucleated cells per cubic millimeter, 54% neutrophils, 22% lymphocytes, and 24% monocytes, with a total protein level of 83 g/dL
.
The CSF VDRL test was reactive at 1:2; this result supports the diagnosis of neurosyphilis
.

Analysis of the patient's cerebrospinal fluid showed a colorless, non-turbid fluid with 21 nucleated cells per cubic millimeter, 54% neutrophils, 22% lymphocytes, and 24% monocytes, with a total protein level of 83 g/dL
.
The CSF VDRL test was reactive at 1:2; this result supports the diagnosis of neurosyphilis
.

The answer is already obvious, the patient's diagnosis: neurosyphilis
.

The answer is already obvious, the patient's diagnosis: neurosyphilis
.
The patient's diagnosis was: neurosyphilis
.

So why does neurosyphilis cause hearing loss in patients? What are the possible pathophysiological processes?

So why does neurosyphilis cause hearing loss in patients? What are the possible pathophysiological processes? So why does neurosyphilis cause hearing loss in patients? What are the possible pathophysiological processes?

There is evidence of hematogenous spread of Treponema pallidum throughout the body and possibly into the temporal bone
.
In the later stages of syphilis, CSF enters the inner ear as it passes through the inner auditory canal or the cochlear aqueduct
.
The inner ear is immunoreactive and Treponema pallidum should elicit humoral antibody responses and activate innate immunity through proinflammatory cytokines and CD4+ and CD8+ T cells
.
All the necessary components of this response are located in the inner ear in the area of ​​the endolymphatic sac
.

There is evidence of hematogenous spread of Treponema pallidum throughout the body and possibly into the temporal bone
.
In the later stages of syphilis, CSF enters the inner ear as it passes through the inner auditory canal or the cochlear aqueduct
.
The inner ear is immunoreactive and Treponema pallidum should elicit humoral antibody responses and activate innate immunity through proinflammatory cytokines and CD4+ and CD8+ T cells
.
All the necessary components of this response are located in the inner ear in the area of ​​the endolymphatic sac
.

Does continued glucocorticoid use after syphilis diagnosis and treatment increase the likelihood of hearing recovery?

Does continued glucocorticoid use after syphilis diagnosis and treatment increase the likelihood of hearing recovery? Does continued glucocorticoid use after syphilis diagnosis and treatment increase the likelihood of hearing recovery?

Dr.
Harris: Patients with symptoms of various forms of rapidly progressive sensorineural hearing loss are often treated early with high-dose corticosteroids, sometimes intratympanic corticosteroids, which may reverse hearing loss and dizziness
.
Treatment of neurosyphilis with antibiotics alone may lead to infection control, but glucocorticoids are required to reduce inflammation in the inner ear and prevent irreversible fibrosis and osteogenesis
.

Dr.
Harris: Patients with symptoms of various forms of rapidly progressive sensorineural hearing loss are often treated early with high-dose corticosteroids, sometimes intratympanic corticosteroids, which may reverse hearing loss and dizziness
.
Treatment of neurosyphilis with antibiotics alone may lead to infection control, but glucocorticoids are required to reduce inflammation in the inner ear and prevent irreversible fibrosis and osteogenesis
.
antibiotic infection

Therefore, subsequent glucocorticoid therapy will be considered if the patient has received long-term antibiotic therapy consistent with the recommendations of the Centers for Disease Control and Prevention
.

Therefore, subsequent glucocorticoid therapy will be considered if the patient has received long-term antibiotic therapy consistent with the recommendations of the Centers for Disease Control and Prevention
.
prevent

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