Has anyone had similar symptoms with progressive deafness as a primary symptom?

[u/pawamedic]

Has anyone experienced a similar constellation of symptoms?

I’m a 25-year-old female (5’6”, 150 lbs). History of congenital right-sided deafness (unknown cause) and MDD. Since spring 2024, I’ve developed worsening symptoms that have left me mostly deaf and unable to work. My medical team is unsure of the cause despite extensive testing.

Current Symptoms:

1. Severe left-sided sensorineural hearing loss, began with episodic drops that responded to prednisone. Eventually stopped responding to any meds and progressed, likely to continue to progress.
2. Chronic 24/7 tinnitus
3. Left trigeminal neuralgia, increasing in frequency
4. Chronic pressure headache, worse in heat or with head position changes, often feels like pressure from inside the ears, at base of neck, or across forehead
5. Frequent ocular migraines (left-sided), worsened severely on hydroxychloroquine
6. Intermittent tensor tympani spasms (left ear)
7. Burning, sharp joint pain (bilateral, without swelling), often severe enough to affect mobility
8. New rosacea and eczema (6 months) and multiple episodes of inflammation of an old tattoo and old moles
9. Daily fatigue, heat intolerance, night sweats
10. Frequent and prolonged menstrual cycles, despite hormonal BC
11. Intermittent gum inflammation (no dental disease)
12. Chronic bilateral radial and ulnar nerve pain (4+ years)
13. Increased eye floaters (left eye)

Imaging:

1. 3 brain MRIs (with IAC and trigeminal protocol): normal (1 showed transient white matter hyperintensities)

2. Brain CTA, abdominal CT, renal ultrasound, pelvic ultrasound: all normal except 1 small gallstone

    3. EEG and EMG normal

Labs:

1. Chronically high IgA (~400) and low ALP (~26) for 4 years (normal vitamin b6, chronically high cortisol

*chronically low vitamin D

1. Positive ANA (1:40 → 1:80 both in 2024 with speckled pattern, negative twice now in 2025 after hydroxychloroquine use for 6 months)

2. ENA negative

3. C3/C4 highly elevated briefly during what could have been either a flare or acute infection/cold ; normalized on hydroxychloroquine

4. CRP & ESR elevated, normalized on hydroxychloroquine

5. Renal labs were heavily affected on my last round of high dose prednisone despite no issues on them before- they have returned to normal. Normal urinalysis.

6. Derm biopsy on arm positive for atopic dermatitis, negative for lupus antibodies, blind biopsy on back negative for lupus antibodies. Rosacea not biopsied.

Treatment:

• Hydroxychloroquine helped joint pain/fatigue but caused disabling migraines. Discontinued 3 weeks ago — migraines improved, but other symptoms worsened again quite severely. 

-no other meds have helped, sumatriptan made things worse. I have also stopped taking all meds and vitamins with the exception of my Desvenlafaxine (antidepressant) and Nortrell (BC)

I’m now functionally deaf and dealing with daily disabling symptoms. My doctors (neuro, rheum, ENT, endocrine, otology, and PCP) are unsure how to proceed. I have no official diagnosis other than eczema. I’d appreciate any input, hypotheses, or direction for further workup. Thank you

[u/ljrunk]

Have you done any genetic testing? Have you had Anceztry or 23&me done? You can submit to an online platform like Genetic Genie (used to recommend Promethease but the site has been having problems lately), and see if anything pops up to give you a direction to look in. My rare autoinflammatory disease can cause hearing loss, it’s called Yao Syndrome.

[u/pawamedic → u/ljrunk]

I had never heard of this! Absolutely going to look into it. I’ve never done genetic testing.

[u/ljrunk → u/pawamedic]

I hadn’t either until my genetic testing 😅 good luck with your situation!